THE NATIONAL SOCIETY OF PEDIATRIC HEMATOLOGISTS AND ONCOLOGISTS

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Дорогие друзья и коллеги!

Наступает одно из главных событий этого года – Конгресс SIOP Asia – 2016.

Впервые за всю историю организации конгрессов детских онкологов местом его

проведения стала Москва. Три года назад, когда началась подготовка к данному меро-

приятию, и мы ждали решения о выборе города проведения 10-го юбилейного кон-

гресса, витала лишь слабая надежда на то, что нам удастся встретить всех на россий-

ской земле. И я благодарен руководству азиатского подразделения SIOP за доверие

к нашей работе!

Выбор России для проведения Конгресса неслучаен. Именно опыт

нашей страны, где за относительно короткий промежуток времени удалось

добиться улучшения выживаемости с 5–10 до 80 % и сравняться по этому показа-

телю с европейскими странами, стоял за этим решением. Об этом опыте предста-

вители нашей страны рассказывали в мае 2015 г. в Женеве на специальной сессии

Всемирной организации здравоохранения по детской онкологии. Как было сказано

на сессии, трансляция этого уникального опыта позволит улучшить выживаемость во

многих странах Азии, где сегодня, несмотря на наличие всех современных технологий

(доступных лекарственных средств, оборудования), результаты остаются зачастую не-

удовлетворительными.

Впереди нас с вами ждут интенсивная работа, интересные встречи и профессио-

нальные дискуссии. Надеюсь, что дни 25–28 мая запомнятся нам надолго, и мы сможем

зажечь огонь познания и творчества внутри себя и своих коллег. Девизом московско-

го конгресса SIOP Asia – 2016 стали слова «ДНИ НАДЕЖДЫ». Я бы добавил – Дни на-

деждыи веры! Веры в свою профессию, свои силы, веры в человеческие чувства и в то,

что наши пациенты с каждым днем будут иметь все больше шансов на выздоровление.

Десятая встреча Азиатского подразделения SIOP должна открыть нам новые имена

в профессии, познакомить ученых Востока и Запада, дать каждому из нас дополнительный

шанс профессионально вырасти. Я благодарю вас за сотрудничество и интерес к Конгрес-

су, желаю всем успешного его проведения и счастливого возвращения домой с новыми

идеями и надеждой на будущие встречи.

Всегда Ваш,А.Г. Румянцев

Dear Friends and Colleagues,

One of the most important events in 2016 is coming up. It is the SIOP Asia Congress. And

for the fi rst time it will be taking place in Moscow. Three years ago, when preparations for this

event just started, we were raising our hope that this 10th anniversary meeting would be held

in Moscow and we could welcome everybody on the Russian land. And I want to express my

gratitude to the leaders of SIOP Asia for trusting us!

The choice of Russia as a meeting place is not accidental. Our experience was one of the

reasons for this decision. In a relatively short time interval we were able to increase survival

rates in children with cancer from 5–10 to 80 % which is comparable with leading European

countries. Representatives of our country reported these achievements in May 2015 at spe-

cial WHO session for children’s oncology in Geneva. As it was said at that session, translation

of such unique experience would enable the improvement of survival in those Asian coun-

tries where results are still suboptimal despite of access to up-to-date medical resources.

We are looking forward to intensive work, interesting sessions and professional discus-

sions. I hope our work at this meeting will be very productive and inspirational and you

will keep good memories of these days for a long time. Our motto for this SIOP Asia – 2016

congress is “DAYS OF HOPE”. I would add “DAYS OF HOPE AND FAITH”. Faith in our profession,

in our strength, faith in the fact that our patients will be having more and more chances to

be cured.

I hope The 10th Congress of SIOP Asia will reveal new leaders in our fi eld, bring together

the scientists from the East and the West, and give us opportunities for professional growth.

I thank all of you for your interest in this congress. I wish you a great and productive time

and hope for future collaboration.

Sincerely yours, Alexander G. Rumyantsev

Дорогие друзья!

От лица всей команды Фонда, а также наших волонтеров, друзей,

благотворителей и подопечных я с радостью приветствую всех участ-

ников юбилейного Конгресса SIOP Asia – 2016 в России!

Сегодня в мире достигнут серьезный прогресс в лече-

нии онкологических заболеваний у детей. Этот успех – резуль-

тат постоянного содержательного диалога, в котором участву-

ют врачи, ученые, благотворительные фонды и пациентские

организации.

Нам удается самое важное – говорить на одном языке, делать об-

щие важные шаги в поддержке тех, кто в этом нуждается, и это вселя-

ет уверенность, что вместе мы сможем добиться еще более лучших

результатов и сохранить еще больше детских жизней!

Я убежден, что Конгресс станет еще одной эффективной площад-

кой для обмена передовым опытом и новыми идеями.

Желаю вам плодотворной работы и успехов!

Константин Хабенский,основатель Благотворительного фонда

помощи детям с онкологическими и другими тяжелыми заболеваниями

головного мозга

Dear Friends,

On behalf of our entire Fund’s team, our volunteers, friends, benefactors

and wards, I am glad to welcome all participants of the SIOP Asia – 2016

Congress in Russia!

To date, a signifi cant progress has been achieved in treatment

of children with oncological diseases.

This success is a result of the ongoing substantive dialogue of doctors,

scientists, charitable funds and patients’ organizations. We are able

to manage the most important things – speaking the same language,

doing collective important steps to support those who need it, and

it gives us confi dence that together we can achieve even better results

and save even more children’s lives!

I am sure that the Congress will become a productive forum

for exchanging advanced experiences and new ideas.

I wish you effi cient work and every success!

Konstantin Khabensky, The founder of the charitable foundation

helping children with oncological and other severe deceases of the brain

The Abstract Book was published

with the support of Konstantin Khabensky

Foundation

www.bfkh.ru

Сборник издан при поддержке

Благотворительного Фонда

Константина Хабенского

www.bfkh.ru

ABSTRACT BOOK

При полной или частичной перепечатке ссылка на «Сборник материалов 10го Конгресса Азиатского подразделения Международного

общества детской онкологии» обязательна.

Размещение тезисов по разделам и списки авторов производились согласно заявки.

В статьях представлена точка зрения авторов, которая может не совпадать с мнением редакции.

Сборник издан при поддержке Благотворительного Фонда Константина Хабенского.

C O N T E N T S

DISEASE ORIENTEDHAEMATOLOGYAcute Lymphoblastic Leukaemia Relationship between spontaneous and post treatment apoptotic index and apoptotic proteins to prednisolone response and post induction minimal residual disease (MRD) status in pediatric acute lymphoblastic leukemia (ALL) . . . 14Characteristics and outcome of MLL gene rearrangement positive pediatric AcuteLymphoblastic Leukemia (ALL) – A report on 371 cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15Impact of cytogenetic evolution on outcome of allogeneic hematopoietic stem cell transplantationat posttransplant relapses in pediatric acute leukemia patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15V(D)J recombination excision circles as markers of T- and B-cell immune reconstitutionin patients with acute lymphoblastic leukaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16To a question of treatment and prevention of Clostridium diffi cile-associated diarrheaat patients with oncohematological diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16The effi cacy of ursodeoxycholic acid administration in children with oncohematological diseases. . . . . . . . . . . . . . . . . . . . . . 17Possibilities of overcoming drug resistance of blast cells in children with relapsedof acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17MLL-gene rearrangements prediction by NG2 expression in non-infant childhood acute leukemia . . . . . . . . . . . . . . . . . . . . . 18Presence of MLL gene rearrangements in infant acute leukemia could be predictedby tumor cells’ immunophenotype . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18Minimal residual diesease (MRD) in young adults with acute lymphoblastic leukemia(all treated according to the protocol ALL-MB-Minsk-2010). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19The use of cognitive stimulators in clinic of patients with medulloblastomaand acute lymphoblastic leukaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19Prospects of heterocyclic derivatives of 2-aminothiazine and 2-aminothiazoline and thiourea derivatives in combating with leukemic cell lines and with bone marrow cells of patients diagnosed with all . . . . . . . . . . . 20Haemostasis in children with acute lymphoblastic leukaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20Prognostic signifi cance of minimal residual disease in children with B-line acute lymphoblastic leukemiatreated in the Republic of Belarus on ALL-MB-2008 protocol . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21Epigenetic regulation of γ-glutamyl hydrolase in pediatric acute leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21Estimation of the importance parameters of the hematological analysis and hemostasisin bleeding complications in children with acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22The relationship between electrocardiographic changes and iron metabolismin children with acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22Folate/Vitamin B12 Defi ciency in Children with Acute Lymphoblastic Leukemia: Breaking the Myth . . . . . . . . . . . . . . . . . . . . . 23Lipid abnormalities in children with ALL: a pilot study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23Experience in the use of imatinib in combination with low-dose chemotherapyfor acute Ph-positive lymphoblastic leukemia in children in Uzbekistan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24Role MRD analysis for risk-adapted therapy children with acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24Risk Prediction of Fever in Neutropenic Children with Acute Lymphoblastic Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25Gene expression-based classifi cation and risk stratifi cation of pediatric acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . 25Effi ciency of the ALL-MB-2008 protocol in children and adolescents with acute lymphoblastic leukemia. . . . . . . . . . . . . . . . 26The development of a medical device for L-asparaginase loading into red blood cells . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26Outcome of Paediatric Philadelphia-Positive Acute Lymphoblastic Leukaemia (Ph+ve ALL)Using Aggressive Chemotherapy with Imatinib in India . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27Comparison Between UKALL and Lahore Protocol on Induction Response in Pre B,Acute Lymphoblastic Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27The impact of homocysteine level on methotrexate induced neurotoxicity in children treatedwith acute lymphoblastic leukemia protocol . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28Malignancies in infants: Epidemiologic profi le and outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28Relapses of acute lymphoblastic leukemia in children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28Long-term outcomes of recombinant human erythropoietin therapy in anemic childrenwith acute lymphoblastic leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29Our Patients with ALL – Just a Phone Call Away . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29The experience of carrying out auto-HCT to the patient with Burkitt-type ALL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30Evaluation of risk factors and patterns of CNS complications in childhood haematologicalmalignancies during early phase of treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

Outcomes of childhood acute lymphoblastic leukaemia treatment accordingto the ALL-MB-2008 and ALL-MB-2015 protocols in the Arkhangelsk region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31Clinical signifi cance of genetic changes in childhood T-cell acute lymphoblastic leukemia (ALL).Results of the multicenter group Moscow–Berlin (MB) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31Endoplasmic reticulum stress-modulated miRNome in Jurkat cells: integrative analysis of miRNA targets . . . . . . . . . . . . . . . 32

Myeloid Leukemias, Myelodysplastic and Myeloproliferative Syndromes Long-term follow-up of children with chronic myeloid leukemia after tyrosine kinase therapyat the Republic of Bashkortostan. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33Hematopoietic stem cell transplantation signifi cantly improve outcome of infantswith acute myeloid leukemia carrying translocation t(7;12)(q36;p13) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34Outcome of the pediatric patients with normal karyotype acute myeloid leukemiain Yeungnam region, South Korea: multi-center retrospective analysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34The features of immunological and morphocytochemical diagnosis of acute megakaryoblastic leukemia . . . . . . . . . . . . . 35Morphological characterization of the leukocytes bound to an anti-CD antibody microarrayallows suggesting preliminary diagnosis in acute myeloid leukemia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35Case of successful treatment of acute myeloid leukaemia with extramedular aff ection at child in Tyumen region . . . . . . . 36Myasthenia gravis and chronic myeloid leukemia in a child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36Myeloproliferative disorders and leukemia in newborns with Down syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37Eff ectiveness, toxicity and place of epigenetic therapy in childhood acute myeloid leukemia. . . . . . . . . . . . . . . . . . . . . . . . . . . 37The impact of conditioning intensity on allogeneic stem cell transplantation outcome of childrenand adolescents with acute myeloid leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38High Level of miR-196b at newly diagnosed pediatric acute myeloid leukemia predicts a poor outcome. . . . . . . . . . . . . . . . 38The suppression of CCL2 expression decrease the proliferation of HL-60 cells through downregulationof cyclin D1 via arresting cells at the G1 phase . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39High expression of S100A8 gene is associated with drug resistance to etoposidevia infl uencing apoptic genes expression and poor prognosis in acute myeloid leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39Abandonment and outcome of childhood acute myeloid leukaemiain a tertiary level hospital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40Crosstalk between THP-1 and MSC changes adipocyte and osteoblast diff erentiation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40Prognostic signifi cance of molecular genetic changes in childhood acute myeloid leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . 41Acute myeloid leukemia as a second disease. Belorussian experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41

Lymphomas The effi cacy of cyclosporin in the treatment of infant case with subcutaneouspanniculitis-like T-cell lymphoma (SPTCL) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42Modern aspects of diff erential diagnosis and treatment of large B-cell lymphomaswith mediastinal involvement in children and adolescents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43Cytogenetic adverse prognostic factors in childhood Burkitt lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43Hodgkin lymphoma in young adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44The infl uence of “bulky” disease on survival in patients with Hodgkin lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44Pair linear dependence of prognostic factors with a tumor volume in young adultswith Hodgkin’s lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45Positron emission tomography in diagnosis of malignant lymphomas in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45Intensive therapy children with advansed stage Hodgkin’s lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46Burkitt’s lymphoma in children: Is a second CVP eff ective in critically ill children? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46Еvaluation of morbidity, mortality and survival in children and young adolescentswith malignant lymphomas in Arkhangelsk region in 2003–2014. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47Use of blinatumomab for treatment of early bone marrow relapseof B-cell lymphoblastic lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47The use of Kinesio Taping according to the lymphatic correction techniquein children in oncohematological practice . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48A study of the demographic profi le, type and treatment outcomes in paediatricnon-Hodgkin’s lymphoma at a tertiary care centre in Karnataka, South India. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48Clinical profi le and prognostic factors in children with sporadic Burkitt’s lymphoma:An Indian experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49Strategy of treatment children with relapse/refractory Hodgkin’s lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49Venous thromboembolism in children and adolescents with non-Hodgkin’s lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50

HistiocytosisClinical case of verifi cation of the Langerhans cells histiocytosis diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51Late diagnosis of Langerhans cell histiocytosis (LCH) in children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52Histiocytic infl ammatory response as predictor of relapse in hematological and lymphoid malignancy . . . . . . . . . . . . . . . . . 52

Incidence and clinical spectrum of drug resistant bacterial infections and associated mortality in pediatric hemato-oncology: experience from a tertiary care centre in South India . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53Study of late eff ects and permanent consequences of pediatric onset Langerhans cell histiocytosis . . . . . . . . . . . . . . . . . . . . 53Langerhans cell histiocytosis (LCH) of the female genital tract:A case report and review of the literature. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54Histiocitosis of cells de Langerhans. Series of cases in pediatric population.National institute of Oncology and Radiobiology (INOR) 1980–2015. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54

SOLID NON BRAIN TUMOURSNeuroblastoma Segmental chromosomal alterations have prognostic impact in primary and relapsed neuroblastoma patients . . . . . . . . . 55Stromal Collagen type XI alpha 1 COL11A1 expression in neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56Treatment of patients with neuroblastoma stage 4S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56Curative eff ect analysis of sequential autologous stem cell transplantation on high-risk neuroblastoma in children . . . . . 57Expression of CD133, CD24 in neuroblastoma and prognostic signifi cance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57Incidence and management of chyle leaks following surgery for abdominal neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . 58Characteristics and results of the treatment of patients with intermediate-risk neuroblastoma in the Republic of Belarus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58Outcomes of children with intermediate risk neuroblastoma:Experience from a tertiary cancer centre in India. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59Neuroblastoma with asymptomatic epidural extension: prolonged survival with palliative care . . . . . . . . . . . . . . . . . . . . . . . . 59Treatment of high-risk neuroblastoma: experience of Russian federal centers. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60Epidural compression at the onset of neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60Retrospective comparison of treosulphan/melphalan versus carboplatin/etoposide/melphalan as preparative regimen for autologous transplantation in pediatric neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61Мinimal invasive surgical treatment of children with thoracoabdominal localization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61Combination of chemotherapy and targeted therapy in treatment of very high-risk patientswith neuroblastoma and Ewing sarcoma family of tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62Vascular “Image-defi ned risk factors” in abdominal neuroblastoma that were determined by ultrasound . . . . . . . . . . . . . . . 62Introduction the 131I-MIBG therapy in treatment of patientswith high risk group neuroblastoma in Russian Federation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63

Renal Tumours Results of treatment of children with bilateral nephroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64Renal cell carcinoma in children: report of two cases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64Diagnosis of renal tumours in neonates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65The fi rst experience of nephron-sparing surgery in children with Wilms tumorusing a single laparoscopic access. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66Renal tumors in infants less than 6 months of age . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66Neoadjuvant transcatheter arterial chemoembolization combined with systemic chemotherapyfor treatment of clear cell sarcoma of the kidney (CCSK). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67Inhibition of autophagy in nephroblastoma and the potential therapeutic signifi cance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67Rare pediatric renal tumors: always keep in mind. An experience of one laboratory . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68Five year experience of Wilms tumor at a tertiary care centre, where we stand?A developing country perspective . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68The treatment of Wilms’ tumour, a single institution experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69

Bone Tumours Level of the TFPI-2 mRNA expression and VEGF-A165/VEGF-A189 ratio in pretreatment tumour tissue is anindependent predictor of poor prognosis for pediatric patients with nonmetastatic Ewing’s sarcoma. . . . . . . . . . . . . . . . . . . 70Endoprosthetic reconstruction of knee joint in children and adolescents with primary bone tumorsin Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev . . . . . . 71The role of radiation therapy in treatment of pediatric and young adult patientswith Ewing sarcoma family tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 71Preliminary treatment results of patients with malignant bone in Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72Experience in the treatment of Ewing’s sarcoma of the pelvis in children. East-European Sarcoma Group. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72

Soft Tissue SarcomasExperience of high-dose chemotherapy and autologous hematopoietic stem cell transplantationof pediatric patients with high risk rhabdomyosarcoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73

Results of treatment of soft tissue sarcomas at children during 12-years period . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74Rapidly progressive Kaposi’s Sarcoma in an Iraqi boy received valproic acid:a case report and review of literature. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74Application of punctional gastrostomy at children with sarcomas of soft tissuewith localization on head and neck. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75Interstitial brachytherapy for childhood soft tissue sarcomas:long term disease outcome & late eff ects. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75Childhood non rhabdomyosarcoma soft tissue sarcomas pattern and outcome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76

RetinoblastomaRetinoblastoma: multimodal management in cancer care hospital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77Inhalational ambulatory anesthesia with sevofl urane in children with retinoblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77Intraarterial and intravitreal chemotherapy in the combined treatment in childrenwith group C and D intraocular retinoblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 78Treatment outcome of retinoblastoma with combined systemic, intraarterial,and intravitreal chemotherapy: a single center experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79Expression of transcriptional target of p53 protein in human retinoblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79The eff ectiveness of ophthalmological treatment of recurrent and resistant retinoblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . 80

Liver TumoursMultimodality treatment outcome of hepatoblastoma: our experiencein a resource limited country. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81Surgical options in complex treatment of focal liver lesions in pediatric patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82Clinical experience and outcome of hepatoblastoma in children: a 9-year retrospective study. . . . . . . . . . . . . . . . . . . . . . . . . . 82Assessment of early tumor response to cisplatin monotherapyin patients with standard risk hepatoblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83

Germ Cell Tumours Characterization of germinals pediatric tumors in National Institute of Oncologyand Radiobiology of Cuba (2000–2012) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84

Rare TumoursMalignant melanoma in children: etiology, treatment, and prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85Own experience of treatment of aggressive fi bromatosis at children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86Subcutaneous Ewing sarcoma: an indolent course of disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86Congenital atypical teratoid/rhabdoid tumor of the brain, rhabdoid tumor of the kidneywith heart metastases in a 2-months-old girl. A case report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87Colorectal carcinoma in children and adolescents. Single center experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87Pediatric infl ammatory myofi broblastic tumor: Experience of multicenter cooperation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88Clinical experiences of infantile hepatic hemangioendothelioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88A case of neuroglial dystopia of nasopharynx, pterygomaxillary fossa, submandibular area . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89Rare types of lung tumors in children. Experience of Federal Research Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89Intratissue infrared lazer thermotherapy in the treatment of kaposiform hemangioendothelioma . . . . . . . . . . . . . . . . . . . . 90Desmoplastic small round cell tumor in children and adolescents: single institution study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90

Other Solid Non-CNS TumoursThe nasopharyngeal cancer in children: Epidemiologic aspect and scalable . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91Aggresive fi bromatosis in childhood at the oncologic pediatric serviceof National Oncology Institute of Cuba (2003–2013) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92Results of investigation of main complex of HLA-phenotype at patientswith solid malignant tumours. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92

CNS TUMOURSA case of hepatic veno-occlussive disease (VOD) presented as huge hemothorax afterthe fi rst chemotherapy for childhood medulloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93Low-grade gliomas in children, associated with neurofi bromatosis type I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94Epidemiology of low-grade gliomas in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94Placement of the Ommaya reservoir in narrow and slit-like ventriclesusing a neuronavigation system . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95

The role of adjuvant chemotherapy for radiation therapy of diff usely growing tumorsof the brain stem in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95The responsiveness of pediatric brain tumor patients with poor prognostic factorsto combination treatment with oral temozolomide and radiotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96An eff ective stereotactic radiotherapy for recurrent pilomyxoid astrocytomain the medulla oblongata of pediatric patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96Surgical treatment of low-grade gliomas (LGG) in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97Reirradiation treatment of diff use brain stem tumors in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97High-dose chemotherapy with autologous bone marrow transplantationin the treatment of pediatric brain tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98Epidemiological characteristics of cerebral tumours in children in Nizhny Novgorod region . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98Neurocognitive disorders in children with post cranial fossa tumour . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99Brain tumors infant, experience of pediatric oncology unit in Algiers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99Review on the management & outcome of childhood cerebralor cerebellar low grade glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100Neurological disorders in case of central nervous system (CNS) tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100Preliminary results of treatment according to HIT-MED-Study Guideline 2014in Russian cohort of patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101Results of medulloblastomas treatment in children under 5 years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101Eff ect therapy for medulloblastoma within protocol HIT-2000/2008among children under the age of four years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102Results of treatment of intracranial AT/RT in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103Results of treatment for childhood with anaplastic ependymoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103Radiation therapy and Nimotuzumab in children and adolescents with brainstem gliomas:a 5-year institutional experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104CNS Tumors in Children of Tatarstan Republic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104Evolution of brain tumours treatment results in childhood during 20 years in Voronezh region . . . . . . . . . . . . . . . . . . . . . . . . 105

NON-MALIGNANT HEMATOLOGYPrediction of course of ITP in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106The experience of propranolol use in patients with Kasabach–Merritt syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107The case of successful treatment of right atrium thrombosisand right internal jugular vein in newborn. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107Visceral leishmaniasis in the practice of pediatric oncohematologist. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108Serum interleukin-10 level in childhood immune thrombocytopenia and its rolein predicting the clinical course – a prospective case control study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108Coombs test – easy diagnosis of diffi cult diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109Phenotypic variability in ELANE–related neutropenia from mutated Ala57 residue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109Castleman’s disease in pediatric patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110Lymphomas in primary immunodefi ciency patients: single center experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110Certain ELANE mutations predispose to more severe courseof severe congenital neutropenia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111

HEMATOPOIETIC STEM CELL TRANSPLANTATIONFunctional activity of platelets and spatial thrombin generation after platelet concentrate transfusionin children after hematopoietic stem cell transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112Experience of treatment of chronic graft-versus-host disease usingthe method of extracorporeal photopheresis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113The acute form of “graft versus host diseases” in children: prognostic factorsand treatment of mesenchymal stem cells steroidrefractory form . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113Survival and treatment of children with acute graft versus host disease:an 8-year single-сentre experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114Invasive fungal diseases in adolescents and young adults after allogeneichematopoietic stem cell transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114New HLA-haplotypes as the result of crossover . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115The safety of hematopoietic stem cell apheresis in children with oncological diseaseswith body weight up to 15 kg . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115The role of bronchoscopy in the diagnosis of invasive pulmonary mycosisin children after allogeneic hematopoietic stem cells transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 116The related factors of infl uencing unrelated umbilical blood transplantationon children with Wiskott–Aldrich syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117Second haploidentical stem cell transplantation as a salvage therapy for childrenwith acute leukemia relapsed after fi rst allo-HSCT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117

Cytomegalovirus retinitis in hematopoetic stem cell pediatric transplant patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118The role of bone marrow stromal cells functional characteristicsin post-transplant hematopoietic reconstitution. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118Feasibility and outcome of allogeneic hematopoietic stem cell transplantationwith post-transplant cyclophosphamide in children with acute leukemia. Single centre experience . . . . . . . . . . . . . . . . . . . 119High mixed chimerism in CD3 cell line in patients with AML after allogenic HSCT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119Breastfeeding is not contraindicated in patients undergoing HSCT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120Reduced-intensity versus myeloablative conditioning allogeneic hematopoietic stem cell transplantationfor patients with Hurler syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120Single-center study of outcome of hematopoietic stem cell transplantations:10/10 versus 9/10 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121The effi ciency of the conditioning regimen at hematopoietic stem cells transplantationfrom HLA-matched (10/10) and mismatched (9/10) unrelated donors for childrenwith hematological malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121

TRANSVERSAL THEMESTREATMENT AND CARESurgery Venous access in treating children with cancer: a 6-year experience of a single institution. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123Venous access in the treatment of children with cancer: results of a multicenter study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124Possibilities of endosurgery in diagnosis of tumor diseases of thoracicand abdominal localization at children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124Surgical treatment of children with solid pseudo papillary tumors of the pancreas. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125The use of a single endoscope access at neoplasms of internal genital organs in girls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125Bronchial blockers in pediatric thoracic surgical oncology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125Superselective ophtalmic arterial chemotherapy for retinoblastoma in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126Microsurgical reconstruction of maxilla and mandible in patients with headand neck tumors – pediatric experience . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126Minimally invasive surgery for the splenic lymphoma with splenomegaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127Transnasal endoscopic surgery in children with and without image guidance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127Technology biopsy of tumors of the urinary bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128Specifi c features of ovarian surgery in girls with oncological and hematological diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128Experience of treatment of newborns with tumours from 2003 to 2015. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129Summing up the results of endoprosthesis for children with bone sarcomas:The East-European sarcoma group (EESG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129

Radiation OncologyThe fi rst Russian experience of TomoTherapy applicationfor total body irradiation in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130Helical tomotherapy for Askin’s tumor of chest wall: clinical outcomes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131Intensity-modulated radiation therapy in multimodality therapyfor head-and-neck soft-tissue sarcomas in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131

New Drugs/Experimental TherapeuticsProphylaxis of postanesthetic agitation syndrome in young children with oncological pathology . . . . . . . . . . . . . . . . . . . . . 132

Supportive Care/Palliative CareAtypical presentation, diagnostic challenges and outcome of dengue infectionin immnocompromised children with cancer: experience from India . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133Organization of nutritional support in a children’s cancer hospital(the experience of our centre). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134The results of screening for nutritional status of patients at a childrens’ cancer hospital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134Thrombus depositions as a risk factor for the developmentof catheter-associated deep venous thrombosis in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135Hypercoagulation phenomenon in children with deep venous thrombosis in the setting of treatment of malignant neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135Time-to-antibiotic administration in children with febrile neutropenia: a quality of care initiative. . . . . . . . . . . . . . . . . . . . . . 136Evaluating the eff ectiveness of ketamine plus atropine as anaesthesiafor intrathecal chemotherapy and bone marrow aspiration at hospital, Vietnam . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136Features of enteral nutrition and anorexia overcomingin hematopoietic stem cell transplantation in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137

Patient-controlled analgesia with tramadol in children and adolescents with gastro-intestinal mucositis afterhigh-dose chemotherapy with autologous hematopoietic stem cell transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137Spectrum of microbiological infections and resistance pattern in pediatric oncology patients:experience from a tertiary care center in North India. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138Successful emergency helicopter transfer for a boy of mediastinal lymphoblastic lymphoma complicatedwith reexpantion pulmonary edema and cardiac tamponade . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138Indicators of the Russian infrastructure of inpatient pediatric palliative care with malignancies . . . . . . . . . . . . . . . . . . . . . . . 139Cytomegalovirus (CMV) viraemia and disease in children with haematological malignancies undergoingconventional chemotherapy: a study from a referral cancer centre in India . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139Disparities in the global landscape of palliative care clinical trials. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140Evaluation of chemotherapy-induced oral mucositis in pediatric oncology hospital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140Percutaneous endoscopic gastroscopy in children with cancer and organic CNS disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141

PsychosocialThe organization of the social help to the families having a child with an oncological diseaseat the Medical and Rehabilitation Scientifi c Center “Russian Field” . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142Testing of cognitive, emotional and behaviour disorders in childrenwith acute lymphoblastic leukaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143Social isolation as a long-term psychological side eff ect of bonemarrow transplantation in children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143Clinical psychological rehabilitation program of the Federal Research Center of Pediatric Hematology,Oncology and Immunology named after Dmitry Rogachev for children with oncological diseases . . . . . . . . . . . . . . . . . . . . 144To the justifi cation of the investigational approach to factors of psychological adjustmentto hematopoietic stem cell transplantation in pediatric oncology/hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145Needs for provision of information and communication in familieswith adolescents in pediatric oncohematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145Level of psychological trauma in mothers of children with cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 146Evaluation of burnout syndrome in pediatric hematologists oncologists. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147Reproductive behavior of families with children-cancer survivors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147Psychological service development of PBHF of SO ODCH 1 Children’s Oncologyand Hematology Centre . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148The stigma of cancer in developing countries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148Psychiatric issue of “medical stress” of parents in pediatric oncology/ hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148Subculture groups of children in medical institution in aspect of interactionwith parents communities and public organizations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149

NursingThe usage of central venous access devices to improve the quality care in children,treated with intensive chemotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150How to organize the best nurses care of venous catheter . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151The usage of innovative methods of sanitary disposal of the surgical unit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151

EPIDEMIOLOGYPerfection of the organizational-methodological approaches to carefor children with cancer in the Russian Federation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152Spontaneous tumor rupture in children: a rare event with fatal outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153Childhood solid tumors incidence in the Kyrgyzstan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153Childhood cancer morbidity in the Republic of Mordovia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154Molecular epidemiology of persister populations of opportunistic bacteria isolatedfrom children with hematological malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154Epidemiology of ovarian neoplasms in girls of diff erent age groupsin the Republic of Bashkortostan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155The incidence of the most common malignant tumors of childhoodin the Chelyabinsk region for the 8-year period . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155Survey on pediatric cancer patients in Kanto-Koshinetsu area after the electionof Childhood Cancer Core Hospitals in Japan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156The positive impact of the protocols of empirical anti-infective therapyon the drug resistance index (DRI) and pharmacoeconomics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156Incidence, structure and ethnicity of solid tumors in children of the Republic of Dagestan . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157

MODERN DIAGNOSTIC APPROACHESThe role of positron emission tomography in staging and determining treatment tacticsin children with sarcomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158Eye tracking method as a diagnostic tool for assessment of oculomotor controland visual perception in patients with medulloblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159Adenovirus hepatitis. Diagnostic features and diff erences of tumor formation and metastasis. Case report . . . . . . . . . . . . 159The results of serologic and bacteriologic examinations of bronchoalveolar lavagefor fungal infection in immunocompromised patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160The use of multislice computed tomography for the diagnosis of minimally invasive fungal pneumoniain children with hematologic malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160The frequency of detection of herpes group viruses in children with oncohematological diseases . . . . . . . . . . . . . . . . . . . . 161

LATE EFFECTSMusculoskeletal sequelae of childhood solid tumours survivors, treated with intensive chemotherapy,surgery and radiation therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162Secondary tumours in children of the Nizhny Novgorod region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163Recovery of motor skills in patients with malignant neoplasms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163Application of cosmetics in the rehabilitation of childrenwith skin manifestations of chronic GVHD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164Assessment of gonadal function in girls after allogeneic transplantwith treosulfan-based conditioning regimens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164Study on late eff ects of treatment of oncological diseases in children:the experience of a specialized clinic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165Functional and ultrasound diagnosis in the assessment of late toxicity in children with oncological diseases . . . . . . . . . . 165Microelemental homeostasis in patients with oncological diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166The structure of the functional impairment in adolescent survivors of childhood cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166Dental health of the patients cured of malignant neoplasms in childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167Carbohydrate-lipid metabolism disorder in patients with oncohematological pathology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167Second tumours in patients cured of malignant neoplasms in childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168

CHILDHOOD CANCER INTERNATIONAL

(FOUNDATIONS/PARENTS/SURVIVORS)Keep up your spirit to conquer cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169Indonesian childhood cancer foundation’s succesful programs and initiatives for survivors. . . . . . . . . . . . . . . . . . . . . . . . . . . . 169The central venous catheter (CVC) checklist for anaesthesiologists . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170Photohromotherapy alternative method for treatment hemangioma in infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170The greif care programs in Children’s Cancer Association of Japan (CCAJ) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171Client – centered psychological assistance to children with cancer and their families. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171“Forum of Winners” as a mean of rehabilitation of teenagers and young adults who suff ered serious illness . . . . . . . . . . . . 172

OTHER TREATMENT AND CARESafety of cyclophosphamide infusion in the outpatient setting in pediatric patientswith malignancies: a retrospective review . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173Rehabilitation of children with onco-hematological diseases at Kirov Health Resort “Avitec” . . . . . . . . . . . . . . . . . . . . . . . . . . 174The effi cacy of frozen-thawed irradiated washed erythrocytes in children with hematological malignancies . . . . . . . . . . 174Neurological outcomes in children with extracranial solid neoplasmsby the age of 24 months . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175Effi ciency of photodynamic treatment for recurrent solid tumors in children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175Metronomic chemotherapy with vinblastine, cyclophosphamide, methotrexateand celecoxib in progressive or relapsed childhood cancers. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176Clinical features, predictors and outcome of posterior reversible encephalopathy syndromein children with cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176Study of anticoagulant eff ect of LMWH in children with cancer and thrombosis using Thrombodynamics,aPTT and anti-Xa activity. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177The case of clinical emergency reinfusion of red blood cells in massive blood loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177The results of treatment of intraoperative blood loss in pediatric oncology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178The remedial treatment of children with malignant solid tumoursat an early postoperative stage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178Assessment of patient awareness regarding chemotherapy and related supportive medicationsin pediatric cancer patients in pediatric oncology hospital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179

Assessment of patient awareness regarding chemotherapy side eff ects in pediatric cancer patientsat a pediatric cancer hospital. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179Modern methods of venous access in pediatric oncology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180

Winners of SIOP Asia 2016 Congress Scholarships. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181

D I S E A S E O R I E N T E D

HAEMATOLOGY

SOLID NON BRAIN TUMOURS

CNS TUMOURS

NON-MALIGNANT HEMATOLOGY

HEMATOPOIETIC STEM CELL TRANSPLANTATION

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A B S T R A C T N O . : O P - 0 6 3

Relationship between spontaneous and post treatment apoptotic index and apoptotic

proteins to prednisolone response and post induction minimal residual disease (MRD)

status in pediatric acute lymphoblastic leukemia (ALL)

Bhatia Prateek, Singh Ankita, Trehan Amita, Bansal Deepak, Bhatia Alka, Singh Sachdeva Man Updesh, Jain Richa

Graduate Institute of Medical Education and Research, Chandigarh, India

Key words: ALL, apoptosis index, apoptotic proteins, MRD, pediatric

Introduction. Despite in vitro studies on drug chemo, there is still controversy regarding the significance of apoptosis during remission induction therapy especially in pediatric ALL.

Aim. A Prospective study, involving 30 paediatric ALL cases was done to note clinical relevance of in vivo apoptosis in our cohort of ALL cases.

Materials and methods. All 30 cases were subjected to Annexin V/PI based staining to detect degree of apoptosis (AI) at day-0 and day-35 post induction chemotherapy. In addition,

apoptotic protein expression using fluorescently labelled Bax and Bcl2 antibodies was studied at day-0 and their RFMI ratios calculated. The samples were run on a LSR-II BD cytometer.

Since five cases left treatment after day 8–15 of therapy, hence day 35 marrow, MRD and AI day-35 data was only available in 25 cases for analysis.

Results. There were 22 (73%) male and 08 (27%) female cases with a M: F-2.75:1. The mean age was 5.1 years. Based on TLC, age and immunophenotype at diagnosis, 21/30 (70 %)

were in standard risk, 5/30 (17 %) intermediate and 4/30 (13 %) in high risk category. Only 5/30 (17 %) were T-ALL and rest 25/30 (83 %) were B-ALL. Day 8 absolute blast count was

more than 1000/ul in 7/30 (23 %; poor responders) and less than 1000/ul in 23/30 (77 %; good responders) cases. Post induction, day 35 check marrow status was M1 in 23/25 (92 %) and

M2 in 2/25 (8 %) cases. Day 35 MRD results were available in 21 cases and 5/21 (24 %) had a high MRD of more than 0.1 %. AI-Day 0 ranged from 0.9–16.6 % with a mean ± SD of

5.90 ± 4.5 % and a median of 4.50 %. AI-Day 35 ranged from 1.4–62.8 % with a mean ± SD of 19.64 ± 17.39 % and a median of 14.0 %. The Bax/Bcl2 ratio ranged from 0.2–3.5 with

a mean of 0.83. The ratio was anti-apoptotic i.e. 1 in only 7/30 (23 %) cases. A significant association was noted between low AI at day-0 and a low Bax/Bcl2 ratio with a favourable

MRD status day-35 (P = 0.013 & P = 0.0002 respectively).

Conclusion. Our study results shed further light on role of apoptosis in pediatric ALL and are in accordance with other studies from west. Despite low AI at day-0 being associated

with an unfavourable phenotype, it is useful marker to predict favourable low MRD at day 35. An anti-apoptotic Bax/Bcl2 ratio too suggests a favourable response to induction

chemotherapy. However, studies on larger cohort of cases especially involving those that have disease relapse will help to draw meaningful conclusions regarding usefulness of

apoptosis assessment as a prognostic marker in pediatric ALL cases.

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HAEMATOLOGY

ACUTE LYMPHOBLASTIC LEUKAEMIA

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A B S T R A C T N O . : O P - 0 7 9

Characteristics and outcome of MLL gene rearrangement positive pediatric Acute

Lymphoblastic Leukemia (ALL) – A report on 371 cases

Suleimman Ahmad Al-sweedan, Rafat Jafri, Khawar Siddiqui, Ali Alahmary,

Ibrahim Ghemlaz, Naemah Farhan, Amal Al-Seraihi

King Faisal Specialist Hospital & Research Center

Key words: leukemia, MLL, outcome

Introduction. The presence of MLL gene rearrangement (11q23) in children’s Acute Lymphoblastic Leukemia is associated with a dreadful outcome.

Aim. To review clinical characteristics and treatment outcome.

Materials and methods. This is a retrospective study of 371 cases where we compared 13 MLL-gene rearrangement positive ALL patients with 358 negative, treatment naïve, non-

infantile patients (age at diagnosis ≤ 14 years), treated at our hospital between 2005 and 2014.

Results. Our MLL positive group had a median age at diagnosis of 5.37 Years (min: 2.08 – max: 12.64) in contrast to non-MLL group’s 4.68 Years (min: 1.01 – max: 14.75) with

p-value: 0.238. At diagnosis, 3 (23 %) were ≥ 10 years in MLL positives vs 50 (14 %) in others. There were 7 (53.8 %) males amongst positives vs 210 (58.7 %) amongst negatives.

Median WBC count (109) in MLL positive patients was 35.32 (min: 3.09 – max: 220.58) with 2 (15.4 %) had count > 100K vs 11.4 (min: 0.68 – max: 923) and 46 (12.8 %) > 100K in

others. Amongst MLL positives, at day 14 BM, 12 (92.3 %) were M-1, and 1 (7.7 %) was M-3 compare to 314 (90.5 %) M-1, 18 (5.2 %) M-2 and 15 (4.3 %) M-3 in others. There were 9 (69.2

%) CNS-1 and 4 (30.8 %) CNS-2 in MLL positives vs 302 (84.6 %) CNS-1, 47 (13.2 %) CNS-2 and 8 (2.2 %) CNS-3 in MLL negatives. MLL positives were represented by B-Cell in 10 (76.9 %)

and T-Cell in 3 (23.1 %) in contrast to 298 (83.2 %) B-cell, 22 (6.1 %) Biphenotype and 38 (10.6 %) T-Cell in MLL negatives. Relapse rate in MLL positive group was 7.7 % (n = 1)

compare to 12 % (n = 43) in MLL negatives (P= 1.000). 2 (15.4 %) deaths were recorded in MLL positives vs 27 (7.5 %) amongst MLL negatives; p-value = 0.270. With a median

follow-up of 64 months, fi ve years Overall Survival (OS) was 0.846 ± 0.100 vs 0.915 ± 0.017 (P = 0.221) and Event Free Survival was 0.846 ± 0.100 vs 0.832 ± 0.023 (P = 0.757) in

MLL positives and negatives respectively.

Conclusion. This report shows that no single factor was associated with superior outcome as patient’s age at diagnosis and gender, WBC count, CNS status, Day 14 BM status and

Immunophenotype did not signifi cantly aff ect the probability of OS and EFS.

A B S T R A C T N O . : O P - 0 9 8

Impact of cytogenetic evolution on outcome of allogeneic hematopoietic stem cell

transplantation at posttransplant relapses in pediatric acute leukemia patients

T.L. Gindina, N.N. Mamaev, E. Nikolaeva, O.A. Slesarchuk, K.A. Ekushov, O.V. Paina,

A.S. Borovkova, L.S. Zubarovskaya, B.V. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia

Key words: pediatric acute leukemia, allo-HSCT, cytogenetic evolution, prognosis

Introduction. Cytogenetic abnormalities at diagnosis have signifi cant impact on acute leukemia outcome in both pediatric and adult patients (pts). Moreover, cytogenetic evolution

is considered to be associated with higher incidence of relapse and resistance to chemotherapy. Recent study of Yausa et al., 2013 showed that cytogenetic evolution was an important

predictor of treatment resistance after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in adult acute myeloid leukemia (AML).

Aim. The aim of our work to estimate the prognostic signifi cance of cytogenetic evolution in the pediatric acute leukemia patients relapsed after allogeneic hematopoietic stem cell

transplantation.

Materials and methods. Thirty-seven children and eighteen adolescents with diagnosis of AML (n = 29) and ALL (n = 26) underwent allo-HSCT in complete remission (n = 25) or in

active disease (n = 30) at our University from 2009 to 2015. They were retrospectively reviewed in order to analyze the cytogenetic evolution patterns at posttransplant relapse of acute

leukemia.

Results. Median follow up was 482 days (40–1861). Overall survival (OS) estimated with Kaplan–Meier method was 32 % (95 % CI 20–45) at 2 years. Related transplantation was

performed in 16 (29 %) pts, unrelated – in 21 (38 %) pts, but haploidentical – in 18 (33 %) pts. Before allo-HSCT normal karyotype was revealed in 4 (7 %) pts, one chromosomal

abnormality – in 26 (47 %) pts, two abnormalities – in 4 (7 %) pts, and three or more abnormalities - in 21 (38 %) pts. At posttransplant relapse 36 (65 %) pts demonstrated gain

or loss of chromosomal abnormalities compared to original karyotype, 2 (4 %) pts showed totally diff erent karyotypes, whereas 17 (31 %) pts had no karyotypic changes. Based on

obtaned data, two groups of pts were formed. The fi rst one consisted of pts with cytogenetic evolution at posttransplant relapse, whereas the second group included pts without

karyotype changes. Our study showed, OS to be inferior for both pts transplanted in active disease (26 % vs 40 %; P = 0.028) and with cytogenetic evolution of leukemic clone

(25 % vs 50 %; P = 0.044). Other prognostic factors e.g. patient sex, age, leukemia type, stem cell source, the number of transplanted CD34+ cells and conditioning regimen were not

associated with OS. In multivariate analysis, the independent predictors of OS were the disease status at transplant (HR – 2.05; 95 % CI: 1.07–3.94; P = 0.03) and the cytogenetic

evolution (HR – 2.21; 95 % CI: 1.01–4.85; P = 0.04).

Conclusion. The main predictors of OS in pediatric acute leukemia patients may be the disease status at allo-HSCT as well as the cytogenetic evolution of leukemic clone.

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D I S E A S E O R I E N T E DA B S T R AC TTO P I C

A B S T R A C T N O . : O P - 1 2 3

V(D)J recombination excision circles as markers of T- and B-cell immune reconstitution

in patients with acute lymphoblastic leukaemia

I.V. Obraztsov1, M.A. Gordukova2, E.V. Kononova3, E.V. Tsvetkova1, I.Y. Tomilin4, A.P. Prodeus2, A.G. Rumyantsev1 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Speranskiy Children’s Hospital № 9, Moscow, Russia; 3I.M. Sechenov First Moscow State Medical University, Russia; 4Morozov Children’s Hospital, Moscow, Russia

Key words: ALL, TREC, KREC, immune reconstitution, MB-2008, MB-2015, V(D)J recombination, T-cells, B-cells

Introduction. Acute lymphoblastic leukaemia (ALL) is the most frequent haematologic malignancy in childhood. Modern chemotherapy protocols enable a high probability of

remission; however an immune reconstitution can be insuffi cient. T-cell receptor excision circles (TRECs) and kappa-deleting recombination excision circles (KRECs) are circular DNA

segments generated during V(D)J recombination process in T- and B-cells. Our innovative approach allows quantifying TRECs and KRECs in order to assess thymic and bone marrow

output in ALL patients who undergo specifi c treatment.

Aim. To investigate dynamics of T- and B-cell neogenesis and immune repertoire in ALL patients during chemotherapy (primary patients from ALL-MB-2015 study) and long after

(intermediate risk patients from ALL-MB-2008 study) by quantifi cation of TRECs and KRECs.

Materials and methods. 61 (31 boy, 30 girls, mean age 6.8 years) long-term (remission duration – 33 ± 4.3 months) follow-up (FU) patients and 41 primary patients (21 boy,

20 girls, mean age 6.2 years) participated in our study. We performed TREC/KREC quantifi cation once in long-term FU patients; assay was held on induction days 0, 8, 15, 36 and prior

reinductions in primary patients. TRECs and KRECs levels were evaluated by multiplex quantitative rtPCR. Statistics were built in SPSS 19 (IBM, USA) software.

Results. We discovered a strong signifi cant (Pearson’s η = 0.81, P < 0.01) correlation between TREC and KREC levels in a long-term FU set. Surprisingly, no coordination between

TRECs (η = 0.11) or KRECs (η = 0.01) and remission duration was found. We also estimated the impact of diff erent therapy regimens according to randomizations of MB-2008

trial on T- and B-cell reconstitution in a long-term FU set. TRECs and KRECs (P < 0.1 and P < 0.05) were lower in patients who had not obtained L-asparaginase (L-ASP (-)) during

induction cycle; in high-dose methotrexate (MTX) set (P > 0.5 and P < 0.1) and in irradiation set (P < 0.05 and P < 0.1) in comparison to L-ASP (+), low-dose MTX and standard

intrathecal administrations, respectively. We evaluated the importance of therapy options mentioned above as well as remission duration for TRECs and KRECs levels dispersion by

nearest neighbor algorithm. The variables have an equal importance (β = 0.27; 0.26; 0.23 and 0.23, respectively) for TRECs levels dispersion; on the other hand, cranial irradiation has

a highest importance (β = 0.80) for KRECs levels dispersion. We have also estimated dynamics of TRECs and KRECs levels in primary ALL patients. KRECs level increases (P < 0.05) after

cytoreduction phase with subsequent progressive profound suppression of B-cell neogenesis.

Conclusion. Our preliminary fi ndings show that T- and B-cell reconstitution rates are coordinated, however, TRECs and KRECs levels are not correlated with remission duration in

long-term FU patients. Therapy options (L-ASP in induction cycle, high-dose MTX or cranial irradiation) have an equal importance for T-cell neogenesis prediction. Cranial irradiation

at the end of consolidation has the highest importance for B-cell reconstitution. Remission duration is equally important predictor for T- and B-cell neogenesis. Use of L-ASP enhances

both T- and B-cell reconstitution, while cranial irradiation causes depression of T- and B-cell neogenesis. High-dose MTX also causes suppression of B-cell reconstitution. T- and B-cell

neogenesis dynamics during specifi c treatment need further investigation.

A B S T R A C T N O . : P - 1 2 4

To a question of treatment and prevention of Clostridium diffi cile-associated diarrhea

at patients with oncohematological diseases

O.P. Tolmacheva, Yu.P. S’emshikova, S.V. Ovanesiyan, S.Yu. Umnova, E.V. Ursulenko, N.N. Martynovich

Irkutsk Regional State Clinical Children’s Hospital, Irkutsk, Russia

Key words: complications during treatment of oncohematological diseases, pseudomembranous colitis, vancomycin, metronidazole, survival

Introduction. Modern treatment of oncohematological diseases at children supposes high-dose chemotherapy, that can provoke in a lot of cases aff ection of diff erent organs

including GI-tract. Character of aff ection is diffi cult, and important role plays the superfl uous microbial growth of potentially pathogenic and pathogenic fl ora. Frequency of aff ection

on intestine on the background of chemotherapy is rather high and in separate cases can be the cause of fatal outcome.

Aim. Prophylactic usage at oncohematological patients anti-anaerobic medications for prevention of pseudomembranous colitis on a background of immunedepression.

Materials and methods. In 2001–2002 we included to the protocol of observation of children with diff erent oncohematological diseases (leukaemias, lymphomas,

lymphogranulomatosis) with diarrhea and enterocolitis the determination in faeces of toxigenic stamms of Clostridium diffi cile by the method of polymerase chain reaction. It was

revealed that in case of development of pseudomembranous colitis, that was diagnosed by special clinical picture (diarrhea, severe condition) and ultrasonic criterions (increasing of

bowel wall), Clostridium diffi cile was found in 100 % of cases. Also, it was revealed that according the results of cultural analysis of faeces, the association with Candida, Klebsiella spp.,

Staphylococcus spp., etc. in high titres (105 and higher) was diagnosed. To the treatment protocol of all patients during the initial signs of intestinal syndrome on the background of

myelodepression we included the anti-anaerobic medications – vancomycin and metronidazole. These medications were also given for prevention of clostridia infection at children

of high group of risk (myelodepression, massive antibacterial treatment) without any signs of intestinal syndrome. Prophylactic treatment at these patients justifi ed by decreasing

of colonizational resistivity of intestine, disturbance of microecosysteme of intestine by anti-tumour drugs and antibiotics and also high incidence of asymptomatic carriage of

Clostridium diffi cile in hole population.

Results. Dynamical observation showed that early intervention of anti-anaerobic medications is signifi cantly decreases severity of course of this type of colitis and it’s time of

duration. During the last 7 years we did not register no lethal cases from complicated pseudomembranous colitis at these patients, just as earlier, before usage of these medications,

severe colitis registered 3–6 times per year, including unfavourable outcome.

Conclusion. Thus, anti-anaerobic therapy in case of myelodepression is eff ective and helpful way for prevention.

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A B S T R A C T N O . : P - 1 2 5

The effi cacy of ursodeoxycholic acid administration in children

with oncohematological diseases

O.P. Tolmacheva, Yu.P. Syemshikova, S.V. Ovanesyan, S.Yu. Umnova, E.V. Ursulenko, N.N. Martynovich

Irkutsk State Regional Children’s Clinical Hospital, Irkutsk, Russia

Key words: toxic hepatitis, hemoblastoses, solid tumors, high-dose chemotherapy

Introduction. The modern treatment of oncohematological diseases in children (leukaemias, nervous system tumours, solid tumors, lymphomas, hystiocytoses, etc.) implies high-

dose chemotherapy entailing drug-induced liver damage in many cases. The frequency analysis of drug-induced hepatitis in children with the pathology in question for the period of

2001–2014 showed that drug-induced hepatitis appeared in the majority of patients. Patients with accompanying viral hepatitis B and C were excluded from the study. In accordance

with the biochemical activity level, medium severity comprised the majority of cases (40 %), low severity – 27 % and high severity – 22 %. In case of high activity, half the patients

developed a protein synthesis (decreased albumin, increased prothrombine time) and a detoxic liver dysfunction (increased bilirubin).

Aim. Administration of ursodeoxycholic acid during chemotherapy to reduce the frequency of grave toxic hepatitis.

Materials and methods. Since 2009, to prevent drug-induced hepatitis over the whole duration of chemotherapy (since its very beginning and during the maintenance course),

ursodeoxycholic acid in the dose of 10–15 mg/kg/d was prescribed for children. Overall, we observed 210 children aged 6 months – 16 years.

Results. The dynamic observation showed that in 54 % of cases, despite intensive chemotherapy, there was no drug-induced hepatitis or the increase in the transaminase level

was insignifi cant over the whole period of monitoring; in 28 % of cases patients developed low severity drug-induced hepatitis and only in 13 % – medium severity drug-induced

hepatitis. Only 5 % of patients had a high severity disease. An increase in total bilirubin levels was registered only in 14 % of patients comprising, on the average, 60 μmol/l.

We reckon that the medium and high severity liver damage might have been caused, despite the preventive measures, by individual features of the metabolic function of the liver.

The ursodeoxycholic acid tolerance was good, without any side eff ects both at the beginning of the therapy and during a long-term drug administration course.

Conclusion. Thus, ursodeoxycholic acid is characterized by a high hepatoprotective and antifi brotic activity and can be widely used both for therapeutic and preventive purposes

in children with oncohematological pathologies.

A B S T R A C T N O . : P - 1 4 3

Possibilities of overcoming drug resistance of blast cells in children with relapsed

of acute lymphoblastic leukemia

N. Batmanova, M. Shervashidze, N. Kulichkina, A. Popa

N.N. Blokhin Russian Cancer Research Center, Moscow, Russia

Key words: leukaemia relapse, drug resistance, bortezomib

Introduction. Relapses of acute lymphoblastic leukemia (ALL) are the main causes of failures and develop in 10–12 % of patients, and in 1–2 % cases is not achieved remission with

the use of modern protocols. Furthermore, not always possible to achieve remission using only chemotherapy. Therefore, search for new drugs overcoming drug resistance in children

with relapsed ALL is a topical problem. One of the drugs, which can modify the sensitivity of tumor cells to chemotherapy is bortezomib (Velcade®).

Aim. The aim of the study was to estimate the frequency of complete remissions in patients with fi rst and subsequent relapse of ALL, as well as in patients with refractory ALL.

Materials and methods. From June 2011 to December 2015 24 patients with relapsed ALL aged 2–21 years (8.5 years) were enrolled in this study. Boys were 18 (75.0 %), girls –

6 (25.0 %). B-cell ALL was in 17 pts (70.9 %), T-cell ALL was in 7 (29.1 %). First relapse was in 16 (66.7 %), initial refractory occurred in 7 cases (29.2 %), one (4.1 %) child had a

second tumor (T-ALL). Isolated extramedullary relapse was in 6 (25.0 %) cases, isolated BM in 9 (37.5 %) and combined in 9 (37.5 %) children. Relapse localized in: isolated BM –

14 (58.3 %), BM and CNS – 3 (12.5 %), skin and testes - 1 (4.2 %), isolated CNS – 1 (4.2 %), CNS and testes – 1 (4.2 %). Very early relapse revealed in 3 (12.5 %), early in 5 (20.8 %)

children, late in 9 (37.5 %) patients.

Chemotherapy consisted on induction (VCR 1.5 mg/m2 N4, DNR 60 mg/m2 N1, PEG-ASP 2.500 IU/ m2 N4, PRED 40 mg/m2 1–28 days, and bortezomib 1.3 mg/m2 N4); two courses

of post induction chemotherapy: 1) VP-16 100 mg/m2 N5, CPM 440 mg/m2 N5, MTX 5000 mg/m2 N1, bortezomib 1.3 mg/m2 N3 and 2) ARA-C 6000 mg/m2 N4, ASP 6000 IU/m2 N2.

Response after each course estimated by BM results and minimal residual disease (MRD) by immunophenotyping.

Results. Complete remission achieved 20 (83.3 %) pts. Complete response (CR) after induction was in 17 (70.8 %) children. After the second course CR was in 3 (12.5 %) patients.

Three (12.4 %) patients didn’t achieve CR, one (4.2 %) died from sepsis on day 23. Evaluation of MRD after the fi rst course of chemotherapy performed in 14 patients (58.3 %),

and the level of MRD was less than 0.001 % in 11 patients (45.8 %).

The long-term results: DFS in patients with late relapse was 33.3 ± 27.2 %, follow-up 22 months. In patients with early relapse good results were obtained. DFS in patients with

isolated BM was 41.7 ± 30.0 % (follow-up 22 months), in combined relapse was 31.3 ± 24.5 % (follow-up 12 month).

At present 7 patients (29.2 %) are alive in CR. 5 (20.9 %) had late isolated BM relapse B-ALL, two children with late relapse of T-lymphoblastic lymphoma. Four (16.6 %) patients with

BM relapse underwent a SCT, relapse developed in 2 (13.3 %) children, both died.

Conclusion. Thus, the use of bortezomib in combination with standard chemotherapy allowed achieve CR in 83.3 % patients. This therapy is more eff ective for late relapses ALL.

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A B S T R A C T N O . : P P - 1 6 3

MLL-gene rearrangements prediction by NG2 expression

in non-infant childhood acute leukemia

O.I. Illarionova1, E. Matveeva1, Yu.V. Olshanskaya1, E.Yu. Osipova1, A.N. Kazakova1, S.A. Kashpor1, G.A. Tsaur2,

T.Yu. Verzhbitskaya2, T.O. Riger2, L.G. Fechina2, S.A. Rumyantsev1, S.A. Plyasunova1, A.M. Popov1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Children’s Regional Clinical Hospital № 1 / Research Institute for Medical Cell Technologies, Yekaterinburg, Russia

Key words: fl ow cytometry, NG2, MLL-rearrangements, ALL, AML

Introduction. Mixed-lineage leukemia (MLL) gene located in 11q23 region is known to be adverse prognostic factor in childhood acute leukemia (AL) both of lymphoblastic (ALL)

and myeloid (AML) origin. AL associated with MLL-gene rearrangements is a distinct subgroup of hematopoietic tumors with specifi c biological features and poor response to the

conventional chemotherapy. Being widespread in children younger than 1 year, this type of genetic lesion is rather rare event in pediatric patients of non-infant age. Due to the

great variability of MLL partner genes, it could be very useful to predict presence of MLL-rearrangements by any clinical or laboratory parameter. NG2 (neuroglican-2) is specifi cally

expressed by tumor blasts in infants with MLL-rearranged ALL and AML very often, although this is not clearly shown for children older than 1 year.

Aim. To investigate the value of NG2 expression for MLL-rearrangements prediction in children older than 1 year with AL.

Materials and methods. Overall in 1306 consecutive childhood (less than 17 years old) AL cases diagnostic immunophenotyping was performed. Among them 1186 patients

were older than 1 year. NG2 expression was evaluated in B-cell precursor ALL (BCP-ALL, n = 839), AML (n = 204) or AL of ambiguous lineage (n = 54). Thus totally 1097 patients were

included in the study group. NG2 was detected by 7.1 antibody included in diagnostic panels for 4–8-color fl ow cytometry. Samples were considered NG2-positive if more than 10 % of

tumor blasts were positive in respect to internal negative control (T-lymphocytes). MLL-gene rearrangements were assessed by fl uorescence in situ hybridization (FISH), conventional

reverse transcriptase PCR (RT-PCR) and long-distance inverted PCR (LDI-PCR).

Results. NG2 expression at the range from 10 % to 98 % tumor cells was found in 46 of 1097 cases (4.2 %). Among them 24 children (52.2 %) had AML while 22 (47.8 %) represented

ALL. 3 ALL patients also shared the phenotype of biphenotypic leukemia according to EGIL scoring system (M.C. Bene et al., Leukemia, 1995) but haven’t met the MPAL criteria defi ned

by WHO (J.W. Vardiman et al., Blood, 2009). In this group of NG2-positive cases MLL-rearrangements were found in 12 ALL and 16 AML samples while 8 ALL and 7 AML patients were

MLL-germline. For 2 ALL and 1 AML cases genetic data was not available. Thus only 60.0 % of ALL and 69.6 % of AML in NG2-positive group carried rearrangements of MLL gene.

t(4;11)(q21;q23) were the most frequent type of MLL-rearrangement in ALL (7 of 12 cases) while t(9;11)(p22;q23) was prevalent in AML (9 of 16 cases). Percentage of NG2-positive

cells did not diff er in MLL-rearrange and MLL-germline cases both in ALL (P = 0.571) and AML (P = 0.579) subgroups. Lower number of CD10-negative cases was observed in MLL-

negative subgroup of ALL in comparison to MLL-positive ones (37.5 % and 83.3 % respectively, P = 0.104).

In infant AL NG2 is known to have high diagnostic accuracy for prediction of MLL-rearrangements. In our non-infant childhood AL study the probability of MLL-rearrangements in

NG2-positive g was rather low in both ALL and AML groups.

Conclusion. Thus we can’t recommend NG2 as a single marker for MLL-rearrangements prediction in children older than 1 year. Complex immunophenotype analysis could me more

applicable for this purpose in large multicenter trials.

A B S T R A C T N O . : O P - 1 6 7

Presence of MLL gene rearrangements in infant acute leukemia could be predicted by

tumor cells’ immunophenotype

A.M. Popov1, G.A. Tsaur2, T.Yu. Verzhbitskaya2, O.V. Streneva2, E.V. Shorikov2, L.I. Saveliev3,

S.A. Rumyantsev1, L.G. Fechina2 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Children’s Regional Clinical Hospital № 1/ Research Institute for Medical Cell Technologies, Yekaterinburg, Russia;3Ural State Medical University, Yekaterinburg, Russia

Key words: infants, fl ow cytometry, MLL-rearrangements

Introduction. Acute leukemia (AL) in children less than 1 year old is the relatively rare disease with specifi c biological features and poor outcome. It is also characterized by high

incidence of MLL gene rearrangements. Immunophenotype of infants’ leukemia varies due to presence or absence of MLL gene rearrangements.

Aim. To describe of immunophenotype in infant acute lymphoblastic and acute myeloid leukemia (ALL and AML respectively) due to presence of MLL gene rearrangements.

Materials and methods. Totally 540 cases of pediatric AL were studied. 113 patients (59 boys and 54 girls) aged from 5 days to 11 months were included in the study group.

Their data was compared to 427 cases of acute leukemia in older children. Tumor cells immunophenotyping was performed by 6–8-color fl ow cytometry. Detection of various types

of MLL-gene rearrangements was done by fl uorescence in-situ hybridization, reverse-transcriptase polymerase chain reaction (PCR) and long-distance inverse PCR.

Results. ALL was found less frequently in infants than in older children (68.1 % and 86.9 % respectively, pespectively, picant immunophenotypic diff erences were observed

in patients with and without MLL gene rearrangements in both ALL and AML. Number of ALL cases in those tumor cells expressed CD10, CD20, CD45, CD133, CD15, CD65 NG2

signifi cantly varied between MLL-positive and MLL-negative groups (9 % respectively) while OCP for CD10-negativity (76.4 %), CD133-positivity (76.5 %) CD15-positivity (67.7 %)

and CD65-positivity (53.7) was not suffi cient enough. Nevertheless CD10-positive BCP-ALL with MLL-rearrangements diff ered from CD10(+) cases in MLL-germline group. CD10

homogeneous expression was noted frequently in MLL-germline cases than in MLL-rearranged ones (P = 0.001). Although there were found no signifi cant diff erences in CD22-positive

patients’ number, CD22(+)-cells percentage was signifi cantly lower in MLL-positive cases (median 89.9 %, range 25.2–99.7 % and median 99.9 %, range 96.0–99.9 % respectively,

P = 0.003). Thus CD20-negativity, CD10-negativity/low expression, high CD45, CD15, CD65, CD133 and NG2 expression, decreased CD22-expression are immunophenotypic signatures

of MLL-rearranged infant ALL, although NG2 has the highest diagnostic effi cacy. Number of AML cases in those tumor cells expressed CD99, CD133, CD15, CD65, CD4, CD11b, CD61,

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NG2 varied between MLL-positive and MLL-negative groups (P = 0.019, P = 0.012, P = 0.002, P = 0.004, P = 0.005).

Conclusion. Thus immunophenotype of AL in children less than 1 year old diff ers signifi cantly from patients of older age groups. Infants’ ALL and AML immunophenotype varies

greatly due to the presence of MLL gene rearrangements. Complex diagnostic immunophenotyping of infants’ AL allows predicting presence of MLL rearrangements while NG2

and CD11b are the most applicable single markers for ALL and AML respectively.

A B S T R A C T N O . : P P - 1 7 4

Minimal residual diesease (MRD) in young adults with acute lymphoblastic leukemia

(all treated according to the protocol ALL-MB-Minsk-2010)

N.V. Migal, A.S. Lelei, E.A. Stoliarova, L.V. Movchan, R.A. Tarasevich, L.V. Artjuschkevich, O.V. Aleinikova, M.V. Belevtsev

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: minimal residual diesease, young adults, acute lymphoblastic leukemia

Introduction. Introduction of methods of detection MRD in young adults with ALL will allow to expand criteria of an assessment of effi ciency of treatment.

Aim. To assess predictive signifi cance of the minimal residual disease in young adults with ALL on the protocol ALL-MB-Minsk-2010.

Materials and methods. Modifi ed pediatric ALL-MB-2008 – protocol ALL-MB-Minsk-2010 was used in the treatment of young adults (18–29 years) with ALL from January 2010

to December 2015. The study group consisted of 47 young adults (mediana 22.2 years) with B-precursor ALL. All patients (pts) were stratifi cated to intermediate risk groups (ImRG)

according to the protocol ALL-MB-Minsk-2010. Morphological remission (blast cell level below 5 %) was attained by day 36 in 46 (97.9 %) pts. Event-free survival (EFS) was 60 ± 10.3 %,

overall survival – 64.4 ± 12.3 %. MRD was evaluated in bone marrow specimens by 6-colour fl ow cytofl uorometry in young adults on day 15 of induction therapy (n = 37 pts), on day

36 (n = 39 pts). Blast cell level in bone marrow below 0.01 % was regarded as MRD-negative (MRD-negative group pts), while blast cell level of 0.01 % and higher in bone marrow

was regarded as MRD-positive (MRD-positive group pts).

Results. 4 (10.8 %) of 37 pts were MRD- (group MRD-), and 33 (89.2 %) of 37 pts – MRD+ (group MRD+) on day 15. EFS was 100 % and 47.5 ± 15.8 % in group MRD- and group

MRD+ respectively (P = 0.371).

14 (35.9 %) of 39 pts were MRD- (group MRD-), and 25 (64.1 %) – MRD+ (group MRD+) on day 36. EFS (group MRD-) was 85.7 ± 9.4 % and 55.0 ± 12.9 % (group MRD+),

P = 0.03042. Cumulative incidence (CI) of relapse was 0 % (group MRD) and 45.0 ± 13.6% (group MRD+), P = 0.02821. CI of treatment related death (group MRD-) was 14.3 ± 9.7 %

and 0 % (group MRD+), P = 0.0554.

Conclusion. Detection of MRD (day 36 of induction therapy) is necessary for treatment eff ectiveness assessment in young adults of ALL as additional parameter and should be

included into criteria for treatment stratifi cation in thе future protocol ALL-MB.

A B S T R A C T N O . : P P - 1 9 0

The use of cognitive stimulators in clinic of patients with medulloblastoma

and acute lymphoblastic leukaemia

V.N. Anisimov1, M.A. Shurupova2, V.N. Kasatkin1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2M.V. Lomonosov Moscow State University, Russia

Key words: rehabilitation, diagnosis, cognitive stimulators

Introduction. At the moment there is a great need for the automated methods of analysis of higher cognitive functions and motor sphere in the groups of patients with

encephalopathy, organic central nervous system involvement and brain tumours. A number of physiological methods based on the use of automated complexes have proven

themselves to be successful in normotypical groups and in the sphere of higher sporting achievements, furthermore, they were applied in some cases in groups of patients with

pathology (Wells, 2014; Fernandez, 2005; Riederer, 2014; Pogalin et al., 2007).

Aim. Our group is implementing the usage of a line of cognitive stimulators for correction and diagnosis of the condition of children with the stated forms of pathology.

Materials and methods. Cognitive defects in randomized groups of children with acute lymphoblastic leukaemia (ALL) who underwent treatment according to the Moscow-Berlin-2008

protocol as well as children with structural brain disorders associated with medulloblastoma and other tumors of posterior cranial fossa who received treatment within the international

HIT protocol. Methods: a) hardware methods of cognitive functions correction (FitLight, CogniSense, DynaVision); b) interactive correction of cognitive functions: mechanic, computerized.

Results. Declaration on integration of a set of the described methods in the process of diagnosis and correction in randomized groups of children with ALL who received treatment

according to the Moscow-Berlin 2008 protocol as well as children with structural brain disorders associated with medulloblastoma and other tumors of posterior cranial fossa who

received treatment within the international HIT protocol.

Conclusion. We provide a list of techniques and methods that enable to diagnose and perform correction in clinic of pediatric patients with medulloblastoma and ALL. A number

of methods have demonstrated its high effi cacy in normotypical groups, in the fi eld of sport psychology as well as in clinic. A complex of the described methods will improve

the effi cacy of the rehabilitation process, assessment (diagnosis) of motor and cognitive indices associated with the symptoms of the described pathology.

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A B S T R A C T N O . : P P - 1 9 6

Prospects of heterocyclic derivatives of 2-aminothiazine and 2-aminothiazoline

and thiourea derivatives in combating with leukemic cell lines and with bone marrow

cells of patients diagnosed with all

M.A. Orlova1, E.Yu. Osipova2, A.P. Orlov1, T.P. Trofi mova2 1M.V. Lomonosov Moscow State University, Russia;

2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: B-ALL, thiazine and thiourea derivatives

Introduction. Compounds: both fi ve- and six- membered heterocycles, namely, thiazine and thiazoline derivatives and complicated substituted thiourea – were tested for

cytotoxicity against various leukemic cell lines (HL-60, K-562, MOLT-4) and bone marrow cells of patients diagnosed with acute B-lymphoblastic leukemia (B-ALL). The lymphocytes

of healthy donors of the same age range (0-16) were used as a control. The compounds are possessed of eff ector properties (inhibitory of diff erent strength or activatory) respect to

inducible NO-synthase (iNOS), over-expression of which is observed in conditions of leukemic diseases.

Aim. The purpose was to study the eff ect of NO-synthase inhibitors on the leukemic cells and the identifi cation of most perspective compounds.

Materials and methods. We have synthesized new compounds of the above classes which were checked by elemental analysis, 1H NMR and spectrophotometry. Method MTT

assay and leukemic cell lines HL-60, K-562 and MOLT-4 was used to determine cytotoxicity. Tests in vivo of the NOS-inhibitory activity of the compounds were carried out by EPR

spectroscopy with the spin trap on white inbred male mice of the Swiss line, aged 5 months, weighing 27-30 g. The method of fl ow cytometry and confocal microscopy were employed

to spot apoptosis. In vitro NOS-inhibitory activity of the preparations had been found using 3H-citrulline. DMF values determined in vivo on white inbred male mice of the Swiss line.

Results. The dependence in the behavior of leukemic cells and lymphocytes of healthy donors on NOS-inhibitory strength of our compounds is existed. Simultaneously some of

compounds are having radiomodifying eff ect, being radioprotector or radiosesitizer. The increasing of radioprotective strength as well as a rise of NOS-inhibitory activity of compounds

are leading to the enhance of therapeutic index TI (TI = LC50 (for healthy donors cells)/LC50 (for leukemic cells)) when preparations acted on cancer cells of patients with B-ALL.

It seems that the NO reduction leads to a sharp (trigger) change of the impact mechanism on the system, whose behavior after the jump does not depend much on the concentration

of NO (within the margin error) at a new higher level of survival. Thus, healthy cells have a response system to increases and decreases of NO concentration. It can be compared

to a buff er system, whose failure (with certain NO concentration) makes cells viability «jump» to the next higher level.

Conclusion. The role of oxidative stress that is one of the main targets for radioprotectors as well as the role of ER-stress produced by NO (NOS-activity) can be corrected

by represented thiazine-thiazolines and thioureas preparations with diff erent parameters of a dose modifi cation factor (DMF) and of NOS-inhibitory activity.

A B S T R A C T N O . : O P - 2 0 2

Haemostasis in children with acute lymphoblastic leukaemia

E.A. Seregina1, L.I. Zharikova1, M.A. Gracheva1, N.M. Trubina1, H.M. Sepoyan1, A.V. Poletaev1,

T.A. Vuimo1, F.I. Ataullakhanov1–5

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Center for Theoretical Problems of Physicochemical Pharmacology, Moscow, Russia; 3HemaCore LLC;

4Department of Physics, M.V. Lomonosov Moscow State University, Russia;5The Faculty of Biological and Medical Physics, Moscow Institute of Physics and Technology, Moscow, Russia

Key words: acute lymphoblastic leukaemia, thrombosis, coagulation tests, global hemostasis tests

Introduction. The incidence of thrombosis in children with acute lymphoblastic leukemia (ALL) is nearly 40 %. The use of central venous catheters is the cause of 2/3 of all

thrombosis in children with ALL. Fast and accurate assessment of the haemostasis in children with ALL is important and actual problem.

Aim. To investigate the hemostatic state in children with ALL using global hemostasis assays.

Materials and methods. Fifty-four patients aged 1 to 17 years with ALL before the fi rst consolidation phase in ALL-MB-2015 protocol were enrolled in this study. Standard

coagulation tests (APPT, TT, PT, fi brinogen concentration, ATIII concentration, D-dimer concentration), thromboelastography (TEG) and thrombodynamics (TD) were used to assess

coagulation status in all patients before and during the treatment. For all calculations, OriginPro 8.0 (Microcal Software, Northampton, MA, USA) have been used.

Results. TEG parameters and standard tests were in normal (89 %) or in hypocoagulation (11 %) area before the fi rst consolidation. While TD parameters revealed hypercoagulation

in 64 %. The concentrations of fi brinogen and ATIII lowered during the treatment in 87 % of patients. While the other coagulation tests data wasn’t signifi cantly (P > 0.5) changed

during the treatment. Thrombosis occurred in 30 patients (55 %), 5 of whom were symptomatic. ATIII concentration was lowered in 58 % of patients with thrombosis and in 28 %

in group of patients without thrombosis. TD revealed hypercoagulation in 89 % in both groups. While D-dimer level was increased in 43 % of patients without thrombosis and in 15 %

of patients with thrombosis. Similar hypercoagulation by TD parameters but lower ATIII and D-dimer levels revealed reducing lysis potential in thrombosis group. This is confi rmed

by threefold increase in free thrombomodulin concentration in thrombosis group.

Conclusion. Thrombodynamics revealed signifi cant hypercoagulability in patients with ALL. The reduced lysis potential confi rmed by low ATIII, normal D-dimer level and increased

thrombomodulin level is the hypothesis of thrombotic complication in children with ALL.

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A B S T R A C T N O . : P P - 2 0 3

Prognostic signifi cance of minimal residual disease in children with B-line acute

lymphoblastic leukemia treated in the Republic of Belarus on ALL-MB-2008 protocol

E.A. Stoliarova, N.V. Migal, M.V. Belevtsev, O.I. Bydanov, O.V. Aleinikova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: minimal residual disease, acute lymphoblastic leukemia

Introduction. At the moment it is shown that an assessment of the therapy response on a molecular level is a necessary constituent for a prognosis of relapse development as well

as for a resolution of a problem on the therapy intensifi cation in patients with acute lymphoblastic leukemia (ALL) that achieved clinical-hematological remission.

Aim. To evaluate a prognostic signifi cance of minimal residual disease (MRD) in children with ALL that received a treatment on ALL-MB-2008 (de novo) protocol at the period from 2008 to 2014.

Materials and methods. 271 patients of standard and intermediate risk groups with an early B-line ALL aged from 1 to 18 years treated on ALL-MB-2008 protocol, without initial

hyperleukocytosis higher than 100 × 109/l at diagnosis and translocations (4;11), (9;22) that achieved a morphological remission on 36th day of induction therapy, were included in

the study. Bone marrow (BM) sampling was performed on 15th and 36th days of induction therapy on ALL-MB-2008 protocol. Morphological remission was achieved at a content of

blasts of less than 5 % in BM at 36th day of induction therapy. MRD in BM was studied by method of 6-colour fl ow cytofl uometry. The MRD ≤ 0.01 % was assessed as MRD-negative

status, MRD > 0.01 % – as MRD-positive status.

Results. By 36th day of induction therapy the morphological remission was achieved by 263 (97.0 %) of 271 patients. Overall survival for the examined patients was 92.0 ± 1.8 %,

event-free survival (EFS) – 85.7 ± 2.7 %. Sixty eight (27.55 %) patients with ALL had MRD-negative status and 179 (72.45 %) children – MRD-positive status on 15th day of induction

therapy. 64 (94.1 %) and 157 (87.8%) patients that on 15th day were with a negative and positive MRD value, respectively, are in a long-term complete remission. EFS in patients

with MRD-negative value was 95.3 ± 2.7 %, whilst in patients with MRD-negative value EFS was 81.6 ± 4.4 % (P = 0.0432). Cumulative risk of relapses in patients with the negative

value of MRD on 15thday of induction therapy was 3.2 ± 2.3 %, in patients with MRD-positive status - 13.3 ± 4.2 % (P = 0.1285) One hundred fi fty eight (63.71 %) patients had

MRD-negative status and 90 (36.29 %) patients had MRD-positive status on 36th day of induction therapy. 148 (93.7 %) patients with a negative value of MRD and 76 (84.4 %) patients

with a positive value of MRD on 36th day are in a long-term complete remission. EFS in the group of patients with MRD-negative status was 93.4 ± 2.2 %, whilst in patients with

MRD-positive status – 74.9 ± 7 % (P = 0.0077). Cumulative risk of relapses in children with ALL that achieved a molecular remission on 36th day of induction therapy was 3.4 ± 1.7 %,

in MRD-positive patients – 23.8 ± 7.1 %, these diff erences are statistically signifi cant (P = 0.0002).

Conclusion. MRD on 36th day of induction therapy on ALL-MB-2008 protocol is a prognostic factor of the development of ALL relapse in children.

A B S T R A C T N O . : O - 2 1 7

Epigenetic regulation of γ-glutamyl hydrolase in pediatric acute leukemia

Xiao-Wen Chen, Si-xi Liu, Hui-rong Mai, Chang-Gang Li

Shenzhen Children’s Hospital

Key words: γ-glutamyl hydrolase, methylation, miRNA, acute lymphoblastic leukemia, acute myelogenous leukemia, children

Introduction. γ-Glutamyl hydrolase (GGH) regulates intracellular folate and antifolates such as methotrexate (MTX) for proper nucleotide biosynthesis and antifolate-induced

cytotoxicity, respectively. In addition to genetic polymorphism or karyotypic abnormalities, epigenetic alterations like promoter methylation may modulate GGH activity in acute

lymphoblastic leukemia (ALL) cells.

Aim. To explore relationship between methylations of two CpG islands (CpG1 and CpG2) in the GGH promoter region and rGGH mRNA expression in pediatric patients with acute

leukemia (AL). microRNA, emerged as important epigenetic modulators of gene expression, was investigated as well .

Materials and methods. MS-HRM (methylation-sensitive high-resolution melting) and bisulfi te sequencing PCR (BSP) were used to detected methylation change of the two

CpG islands in the GGH promoter region in pediatric patients with acute leukemia (AL) and controls (healthy children and children with immune thrombocytopenia). Simultaneously,

Expression of GGH mRNA andmicroRNA miR-26b-5p was detected by real-time PCR, respectively. Single Nucleotide Polymorphism (SNP) in the 3´-UTR of GGH gene was studied by

HRM and DNA sequencing.

Results. We explored two CpG islands (CpG1 and CpG2) in the GGH promoter region in pediatric patients with acute leukemia (AL) and controls(healthy children and children with

immune thrombocytopenia). Methylations of CpG1 were detected in leukemia cells from 30.9 % (21/68) of patients with ALL and 20.7 % (6/29) of patients with acute myelogenous

leukemia (AML), no methylation detected in the controls. Methylations of CpG2 were detected in the leukemia cells from 44.1 % (30/68) of the ALL patients and 37.9 % (11/29)

of the AML patients, signifi cantly higher than 6.0 % (3/50) of the controls. Our results showed that either methylation of CpG1 or hypermethylation (the methylation level is greater

than 10 %) of CpG2 could signifi cantly reduce GGH mRNA expression in leukemia cells from the ALL and AML patients (ALL-CpG1: n = 38, P = 0.002, ALL-CpG2: n = 46, P = 0.001,

AML- CpG1: n = 18, P = 0.048, AML- CpG2: n = 16, P = 0.090). Furthermore, microRNA, emerged as important epigenetic modulators of gene expression, was investigated as well.

We found expression of miR-26b-5p had a negative correlation with the GGH mRNA in peripheral leukocytes from the controls (Spearman r = -0.526, P = 0.006). miR-26b-5p

expression of leukocytes was higher in peripheral blood than in bone marrow (n = 49, P = 0.002), opposite to the expression of GGH Mrna, Isuggested that miR-26b-5p down-

regulated the expression of GGH mRNA to some extent. In addition, as miRNAs’ binding site, Single Nucleotide Polymorphism (SNP) in the 3´-UTR of GGH gene was studied. There was

no diff erence for allelic frequency of 1385T>C (rs17279558) between the AL patients and the controls.

Conclusion. GGH expression level is partly regulated by epigenetic changes including methylation of CpGs and miRNA. Either methylation of CpG1 or hypermethylation of CpG2 can

signifi cantly reduce GGH mRNA expression in pediatric patients with AL. MiR-26b-5p may down-regulate the expression of GGH mRNA.

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A B S T R A C T N O . : P - 2 1 8

Estimation of the importance parameters of the hematological analysis and hemostasis

in bleeding complications in children with acute lymphoblastic leukemia

N.N. Babaxanova, Kh.Ya. Karimov

Scientifi c-Research Institute of Hematology and Blood Transfusion, Tashkent, Republic of Uzbekistan

Key words: acute lymphoblastic leukemia, hemostasis, children, thrombocytopenia

Introduction. The course of acute leukemias including the earliest stages of the disease are accompanied by the risk of complications associated with hemostasis system disorders.

A timely prevention of such complications calls for the use of reliable markers capable of showing sources of imbalance between coagulant and anticoagulant mechanisms.

Aim. The aim of our study is the assessment of haemorrhagic complications and routine laboratory values of hematologic analyses as prediction markers of hemostasis disorders

in children with acute lymphoblastic leukemia (ALL).

Materials and methods. The main laboratory values of blood and the state of hemostasis were determined in 25 children aged between 6 months and 15 years with newly-

diagnosed ALL. The patients were classifi ed into groups in accordance with the intensity of haermorrhagic syndrome and the severity of thrombocytopenia.

Results. In groups of patients classifi ed in accordance with the stage of haemorrhagic syndrome (HS) (1 – no HS and HS stage I; 2 – HS stages II and III), a performed analysis showed

no clear diff erence between the main laboratory values. Such biochemical parameters of blood coagulation and blood fi brinolytic systems as index of activated patial thromboplastin

time (APTT), prothrombin time (PT), thrombin time (TT), fi brinogen, Hageman-dependent euglobulin lysis time (XIIa/HD-ELT), the number of soluble fi brin monomer complexes

(SFMC) as well as physiological anticoagulants values such as activity of protein C (PC) and antithrombin activity (AT III) did not demonstrate clear changes when comparing the 1st

and the 2nd group of patients.

In cases of deep thrombocytopenia, 38.9 % of patients had clinical signs of hemostasis disorder. There were no clinical signs of hemostasis disorder in the group of children with mild

and moderate thrombocytopenia.

In cases of mild and moderate thrombocytopenia the hemoglobin level was 25 % higher than in cases of deep thrombocytopenia (90.7 ± 5.4 g/l versus 68.0 ± 4.1 g/l; t = 2.6; P < 0.05).

A signifi cant deviation was registered in the concentration of fi brinogen that increased with the aggravation of thrombocytopenia (from 2.7 ± 0.5 to 5.0 ± 0.5 g/l; t = 3.1; positive

D-dimer values are accompanied by thrombocytopenia).

Conclusion. The conducted study showed that the main blood values, coagulation and fi brinolytic system as well as anticoagulants values do not correlate with the severity

of thrombocytopenia and HS and cannot be considered reliable prediction markers of hemostasis disorders in ALL children.

A B S T R A C T N O . : P - 2 2 1

The relationship between electrocardiographic changes and iron metabolism

in children with acute lymphoblastic leukemia

E.D. Teplyakova1, A.A. Savisko1, A.V. Shestopalov1, N E. Laguteeva1, K.S. Aslanyan2

1Rostov State Medical University, Rostov-on-Don, Russia; 2Children Hospital of Region Rostov-on-Don, Russia

Key words: lymphoblastic leukaemia, children, iron metabolism

Introduction. Violation of iron metabolism in children with acute lymphoblastic leukemia (ALL) is a factor that signifi cantly worsens the prognosis of cancer patients increases

the risk of cardiac complications

Aim. The purpose of the study was to establish the correlation between electrocardiographic changes and disorders of iron metabolism in children with ALL during chemotherapy (PCT).

Materials and methods. 36 children with ALL were under the watchful. The age of children was from 2 to 15 years. He received chemotherapy protocol ALL-MB-2008. The survey

was conducted in the dynamics: before chemotherapy, after the induction of remission and after an intensive course of chemotherapy. The comparison group included 32 children

groups I and II of health. The functional state of the myocardium in children with ALL in all groups was assessed by ECG in 12 standard leads alone. The iron content in the blood serum

were determined by the colorimetric method using a set of «Iron-Vital» Vital Diagnostics, Russia. The concentration of ferritin in serum were determined using test-systems “Ferritin

EIA-BEST” (Russia) by enzyme immunoassay.

Results. In the analysis of ECG changes it revealed that the onset of the disease most frequently recorded disorders in automatism– in 50.0 % of children, signs of myocardial

repolarization in 33.3 % of patients, extrasystoles – in 8.3 % people and conduction disturbances in 8.3 % of children. However, after the induction of remission a signifi cant decrease

in the number of patients with ECG signs of disorders in automatism to 33.3 % compared to the previous monitoring period (p in the dynamics of PCT – 47.2 % and 66.7 % respectively

at 2 and 3 of the study period, which may indicate a build-up of metabolic abnormalities in the heart muscle.

In determining the iron content in blood serum of children is determined by its signifi cant increase prior to the PCT by 83.8 %, after induction therapy 24.6 % after intensive

chemotherapy course by 36.6 % compared to the control group.

Conclusion. Joint analysis revealed that at all stages of chemotherapy in children with ALL is set signifi cant interface between disorders in automatism and the level of serum iron,

between the appearance of arrhythmia during treatment and after its completion and the level of serum iron, as well as violations of repolarization in the myocardium at all stages

of PCT, conduction abnormalities during treatment and after, and serum ferritin.Labile plasma iron is iron that not bound to transferrin, easily captured the tissues, including the

myocardium, which is a substance catalyzes the formation of reactive oxygen species. Labile iron toxic eff ect on the heart caused by several mechanisms, including the peroxidation

of lipid membranes, that leads to the release of iron stores and modulating transcription in the nucleus.

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A B S T R A C T N O . : O P - 2 2 2

Folate/Vitamin B12

Defi ciency in Children with Acute Lymphoblastic Leukemia:

Breaking the Myth

Sanjeev Khera, Amita Trehan, Savita Attri, Richa Jain, Deepak Bansal

Postgraduate Institute of Medical Education & Research, Chandigarh, India

Key words: ALL, undernutrition, B12

, folate

Introduction. Vitamin B12

& Folic acid (FA) supplementation in patients with a malignancy are considered counter-productive by making the cell stroma conducive to the

proliferation of the malignant clone. Patients in lower middle income countries (LMIC’s) have greater incidence of malnutrition with vitamin defi ciencies. The need for refraining from

supplementation of FA to patients with a malignancy in LMIC’s has been questioned.

Aim. To evaluate the incidence of vitamin B12

/FA defi ciency in children on therapy for acute lymphoblastic leukemia (ALL).

Materials and methods. Children with ALL on therapy were randomly evaluated for serum B12

and folate levels. Serum B12

less than 211pg/ml and serum Folate less than 2 ng/ml

were taken as defi cient levels. Defi ciency status was correlated to under-nutrition. Weight for age less than -2z score was taken as under-nutrition (WHO).

Results. 70 children with mean age 6.8 yrs (6.03–7.73), including 50 on maintenance and 20 on induction/ consolidation chemotherapy were evaluated. 50 age and sex matched

controls were also evaluated.

Induction/ Consolidation: 6/20 children (30 %) were undernourished. Mean B12

levels were 286.8 pg/ml (227.76–345.84), with 7 (35 %) being B12

defi cient. 2/7 patients were

undernourished. No child had Folate defi ciency, the mean levels being 8.49 ng/ml (5.85–11.13).

Maintenance Therapy: 14/50 children (28 %) were under-nourished. Mean B12

levels were 500.56 pg/ml (419.74–581.38). B12

defi ciency was seen in 5 (10 %) patients, with 3/5 being

under-nourished. Mean Folate levels were 6.61 ng/ml (5.56–7.66), defi ciency being seen in 4 (8 %) patients 3 of whom were under-nourished.

There was no diff erence of B12

& Folate levels when sex, T/ B cell ALL, standard risk Vs high/intermediate risk ALL were compared. Undernourished children had signifi cantly low levels

of Folate (4.59 Vs 7.4; P = 0.0139). This diff erence was not observed with B12

levels.

50 age/sex matched children taken as controls did not have either defi ciency.

Conclusion. Under-nutrition in India is 45.9 % as per National Family Health Survey (NFHS)-3 with reported prevalence of B12

& FA defi ciency in the general population to be 7–33 %

and 20–33 %; with a even higher prevalence in the undernourished. 28 % of our cohort had under-nutrition. We had 5.7 % with Folate & 17 % children with B12

defi ciency. Patients

with coexisting malnutrition had greater B12

/FA defi ciency. Higher B12

defi ciency in induction/consolidation which may be due to higher demands or drug interactions needs further

evaluation. A larger cohort would help conclude and nullify the hypothesis of FA supplementation to cancer patient in LMIC’s. Under-nutrition in LMIC’s remains the main issue to be tackled.

A B S T R A C T N O . : O P - 2 2 4

Lipid abnormalities in children with ALL: a pilot study

Divya R, Amita Trehan, Savita Attri, Deepak Bansal

Postgraduate Institute of Medical Education & Research, Chandigarh, India

Key words: lipids, ALL

Introduction. A relationship between serum lipids and cancer has been demonstrated & lipid abnormalities are considered to be involved in the mechanism of oncogenesis. A low

value of high density lipoprotein cholesterol (HDL-C) and an increase in triglycerides (TG) and low density lipoprotein cholesterol (LDL-C) has been observed in cancers, including ALL.

Aim. To look for abnormalities in the lipid profi le in children with ALL during induction therapy and to evaluate any complications secondary to abnormalities in lipid profi le.

Materials and methods. This was a prospective observational study. Fifty consecutive newly diagnosed children between 1–12 years being initiatecd on therapy for ALL were

analyzed. Relapsed patients were excluded. Serial fasting blood samples were taken for blood sugar, lipid profi le, serum amylase and for liver function tests at 4 time intervals : before

start of therapy, day 14 & day 28 of induction therapy and 2 months into therapy.

Results. Median age of patients was 5 years (IQR 1–12 years). 26 % had a TLC of > 50,000/mm3 at diagnosis. Seven children were > 10 years. Thirty fi ve children were standard risk

and 18 high risk as per NCI criteria

The mean triglyceride (TG) before initiation of therapy was 200 mg/dL (± 95), high compared to population norm of 100 mg/dL (P = 0.001).Triglyceride level decreased during

induction treatment but was higher before, during and after therapy (P = 0.001). No child had any neurological symptoms despite high TG levels.

The mean cholesterol level before initiation of therapy was 144 mg/dl (± 42), signifi cantly lower than the norm of 170 mg/dl. There was no signifi cant change in cholesterol levels

before, during and post induction therapy. The mean LDL-C (low density lipoprotein cholesterol) level at diagnosis was 85 mg/dL (± 35), the population normal being 110 mg/dL

(P = 0.001). Serial LDL-C levels during and after induction therapy remained lower than normal (P = 0.001). The mean HDL-C (high density lipoprotein cholesterol) at admission was

signifi cantly lower: 22 mg/dL (± 12) compared to normal of 60 mg/dL (P = 0.001). Signifi cant increase in mean HDL-C level during induction therapy was noted (30 mg/dL (± 16) vs

22 mg/dL (±12), P = 0.001). However, mean HDL-C during and post induction remained lower than normal (P = 0.001).

Serum amylase was normal at diagnosis. During induction therapy, 4/50 patients had amylase levels greater than 100 U/L, with one having pancreatitis. Two children had diabetic

ketoacidosis during induction therapy. None of them had hyperlipidemia. The mean aspartate transaminase (AST), alanine transaminase (ALT) levels at diagnosis were signifi cantly

high compared to normal population norms. There was a mild increase in AST & ALT levels during therapy.

Conclusion. Lipid profi le in our cohort was abnormal at diagnosis of ALL, with a signifi cantly high TG level and low LDL-C & HDL-C levels. No patient had complications secondary to

these abnormalities. Larger studies are needed to evaluate the cause and eff ect relationship between lipid profi le, malignancy and usage of steroids and asparaginase. Pancreatitis

and hyperviscosity secondary to aberrant lipid parameters induced by asparaginase, steroids and malignancy per se are perchance anecdotal in nature & possibly due to individual

patient variability in response to disease or drug. Epigenetic studies for the same may help in further understanding of this phenomenon.

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A B S T R A C T N O . : P - 2 4 4

Experience in the use of imatinib in combination with low-dose chemotherapy

for acute Ph-positive lymphoblastic leukemia in children in Uzbekistan

S. Ibragimova

Scientifi c Research Institute of Hematology and Blood Transfusion, Tashkent, Republic of Uzbekistan

Key words: children, imatinib, low-dose chemotherapy, acute Ph-positive lymphoblastic leukemia

Introduction. The biological characteristics of leukemic process determines the clinical course of the disease. Of particular diffi culty is the therapy of prognostically unfavorable

variants of ALL in children, such as Ph-positive leukemia. The study COG AALL0031 (2002–2006) for the treatment of children with Ph+ ALL 3 year disease-free survival of patients

receiving imatinib in combination with chemotherapy was 80 % (44 patients), and are held 13ti related transplantation of hematopoietic stem cells (HSCT), the remaining

31 patients received only chemotherapy with imatinib. This study also demonstrated the absence of HSCT advantages compared with chemotherapy in combination with imatinib.

5-year survival rate for patients who received only chemotherapy combined with imatinib was 80 %, a related HSCT – 78 %, unrelated HSCT – 60 %. These data indicate the advisibility

of introducing a combination therapy Glivec in Ph-positive ALL.

Aim. The aim of this study was to analyze the results of the combined use of tyrosine kinase inhibitors, and chemotherapy with low-dose methotrexate in patients with Ph-positive

acute lymphoblastic leukemia.

Materials and methods. 13 patients with Ph-positive ALL who received treatment protocol ALL-MB + Imat in the children’s department of the Research Institute of Hematology

(Uzbekistan) for the period 2009–2013. The average age of the patients was 8.3 ± 1.3, the ratio of sex: 8 boys, 5 girls, median follow-up of 3 years. An initial data: in 3 patients

(23.1 %), enlarged spleen was more than 4 cm in 10 (76.8 %), initial leucocytes count was 30 000 in 7 patients (53.8 %), 30 000 to 100 000 in 13 patients (23.1 %), above 100 000 –

in 3 (23.1 %). All patients with t(9;22) BCR-ABL (Ph+ ALL) of the day 15 gave an additional induction imatinib 300 mg/m2 daily inside. Patients who achieved remission to the 36 day

of treatment, to receive further consolidation chemotherapy according to plan for the intermediate-risk group with low doses of MTX 30 mg/m2/m, with additional lumbar puncture,

with the continuation of receiving imatinib 300 mg/m2 daily inside up to the end of maintenance therapy.

Results. Of the 300 patients with acute lymphoblastic leukemia, studied molecular genetics techniques in the biochip laboratory diagnostics in 13 patients was identifi ed t(9;22)

BCR / ABL p190.

Based on clinical and laboratory data was diagnosed Ph-positive acute lymphoblastic leukemia. Parents of 3 patients (23.1 %) refusal of chemotherapy. 10 patients (76.8 %) received

treatment of protocol ALL-MB + Imat. Early response to the 15th day of therapy determined in 6 patients (60 %). All the 10 patients had clinical remission after induction course

independently of initial data and the response to treatment on the 15th day. 10 patients were relapsed, death in remission determined in 3 patients (30 %). The main cause of mortality

infectious complications were mixed bacterial and fungal etiology. Thus, 7 (70 ± 3 %) patients are in CCR.

Conclusion. Use of tyrosine kinase inhibitors in combination with low-dose chemotherapy has shown high effi cacy in a considerable reduction in toxicity. The use of this protocol

made it possible to outpatient therapy. This signifi cantly improved the quality of life of patients, and resulted in a reduction of infectious complications.

A B S T R A C T N O . : P - 2 5 3

Role MRD analysis for risk-adapted therapy children with acute lymphoblastic leukemia

M. Shervashidze, L. Grivcova, A. Popa

N.N. Blokhin Russian Cancer Research Center, Moscow, Russia

Key words: ALL, MRD, children, risk adapted therapy

Introduction. Although that the overall survival of patients with acute lymphoblastic leukemia has reached 85 % (ALL-BFM-2002), a proportion of them ultimately relapse.

One of impotent reasons of relapses is caused by residual malignant cells that are undetectable by standard diagnostic techniques. Several studies have shown that detection

of minimal residual disease is an independent risk parameter of high clinical appropriateness.

Aim. The aim of our study was to determine minimal residual disease in children with B-precursor ALL (B-ALL), to use this to defi ne risk stratifi cation, to evaluate the signifi cance

of MRD for overall survival, event-free survival (EFS) and disease-free survival (DFS).

Materials and methods. from 2010 to 2015 fi fty pediatric patients with B –ALL were enrolled in ALL-IC-BFM-2009. Median age 5.2 years (range from 1 till 16). Male – 15,

female – 35. The diagnosis was made by standard morphological analysis and by fl ow cytometry immunophenotyping. Forty-four patients had common ALL, 5 pre-B ALL, 1 pro-B ALL.

According to the ALL-BFM-2009 protocol risk classifi cation initially was based on presenting characteristics of patient (sex, age, leukocytosis, genetic translocation and response

on steroid therapy). Eighteen patients had standard risk group (SR), 30 – intermedia risk (IR), 2- high risk (HR). MRD was detected at day 15 of induction therapy. Our studies used

4 and 8-color fl ow cytometry. Although 8- to 10-color fl ow cytometry methods are now becoming standard, our studies began before these were widely available for clinical use. We

couldn’t fi nd the diff erence between results of 4-color and 8-color fl ow cytometry.

Results. Level MRD cted on 29 (58 %) patients, MRD between 0.1 and 10 % had 13 (26 %) patients and MRD ≥ 10 % – 8 (16 %). Thirty six patient had we restratifi cation the risk

status of 5 patients: 2 patients with SR group move to HR and 3 patients from IR to HR. All restratifi cation patient achieved complete remission and still alive without relapse.

Fife years EFS of 92.9 ± 6.9 % (n = 14) for SR, 75.3 ± 13.7 % (n = 26) for IR, and 74.1 ± 16.6 % (n = 10) for HR by the ALL-BFM-2009 risk criteria’s.

Given this stratifi cation 5y-DFS was 92.9 ± 6.9 % (n = 14) for SR, 75.3 ± 13.7 % (n = 26) for IR, and 90.1 ± 9.5 % (n = 10) for HR. It is interesting to note that HR patients received

more intensive of postindaction therapy are showed better DFS results than IR grope which had not changed fi nal risk. There were no cases of induction death.

Conclusion. these results confi rm the importance of MRD detection for risk adapted treatment childhood B-ALL to intensify of postinduction therapy for MRD positive patients on day 15.

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A B S T R A C T N O . : O P - 2 6 9

Risk Prediction of Fever in Neutropenic Children with Acute Lymphoblastic Leukemia

Lutfor Rahman Molla, Chowdhury Yakub Jamal, Momena Begum, Mehnaz Akter, Md. Bani Yeamin,

Md Tanvir Ahammed, SM Rezanur Rahman, Zannat Ara, Rasel Siddique

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Key words: risk prediction, febrile neutropenia, acute lymphoblastic leukaemia

Introduction. Fever in severe chemotherapy induced neutropenic patients is the most frequent manifestation of a potentially lethal complication of current intensive chemotherapy

regime. This study aimed at establishing model predicting the preventable risk of febrile neutropenia in patient of acute lymphoblastic leukemia.

Aim. Identifi cation of risk factors of fever in neutropenic children with acute lymphoblastic leukemia.

Materials and methods. This descriptive observational study was carried in the department of pediatric hematology and oncology department during the period of 1st February

2013 to 31st January 2014. Through purposive sampling 40 neutropenic patients were selected. Their total history and behavioral pattern regarding the supportive management

(neutropenic diet, use of acifl avine solution, nystatin oral solution, mouth wash with povidone iodine),total duration of hospital stay, duration of neutropenia, no of attendant during

hospital stay were recorded. Blood, urine and wound swab culture was done. Nutritional assessment was done according to WHO malnutrition criteria.

Results. Most of the studied child was in induction phase of therapy. The mean hospital stay was 8.56 ± 6.75 days and mean no of attendant with each patient was 2.02 ± 0.65.

Majority of the patient were on neutropenic diet and freshly cooked food (87.5 %). This study shows a large portion (52.5 %) of the studied population did not use acrifl avine as per

advice. It also revealed majority of the child did not use povidone iodine mouth wash (52.5 %) and nystatin (47.5 %) as per advice. A total of 10 patients (25 %) revealed growth of

pathogens. Among them blood culture was positive in 4 patients, urine culture was positive in 3 patients and wound swab culture was positive in 3 patients. This study showed that

major portion (65 %) of the febrile neutropenic child suff ered from malnutrition.

Conclusion. This study showed that majority of the patient did not properly follow the advice regarding behavioral and supportive management. Duration of hospital stay stay and no of

attendant were also high. Malnutrition was present in a large portion of the child. So a large scale multicentre cohort study should be done to validate these fi nding before establishing as risks.

A B S T R A C T N O . : O P - 2 9 1

Gene expression-based classifi cation and risk stratifi cation of pediatric acute

lymphoblastic leukemia

Sun Yanran1, Zhang Han1, Cheng Hao2, Gao Chao1, Wang Qingqing3, Han Jingdong2, Zheng Huyong1

1Beijing Children’s Hospital, Capital Medical University; 2CAS Key Laboratory of Computational Biology; 3Ningbo Health Gene Technologies Ltd.

Key words: acute lymphoblastic leukemia, gene expression, classifi cation, prognosis

Introduction. Acute lymphoblastic leukemia is the most common childhood tumor and remains a leading cause of cancer death in the young. It contains cytogenetically distinct

subtypes that respond diff erently to cytotoxic drugs. Previously, we developed a microarray-based subtype classifi er based on the relative expression levels of 62 marker genes,

and furthermore adopted an advanced fragment analysis (AFA) technique to detect that maker genes, the accuracy of this AFA-based classifi cation attained more than 94 %.

Compared to microarray assays, this technique makes the convenient, low cost and individualized subtype diagnosis of pediatric ALL a reality and is clinically applicable. However, the

prognosis value of this AFA-based classifi cation compare to traditional MICM classifi cation is still unknown.

Aim. To clear the prognostic signifi cance of AFA-based gene expression classifi cation.

Materials and methods. From August 2007 to January 2014, 188 children aged between 9 months and 13 years (median age of 5 years) with newly diagnosed ALL were enrolled in this

study. All patients were diagnosed with ALL using a combination of MICM classifi cations and were treated according to the Beijing Children’s Hospital (BCH) ALL 2003 protocol or Chinese

Children’s Leukemia Group (CCLG) ALL 2008 protocol. The classifi cation of 188 ALL cases according to MICM subtype and the corresponding risk stratifi cation group are shown in table 1.

We chose January 1, 2016 as the end of collection of the patients’ treatment outcomes. Event-free survival (EFS) was defined as the time from the diagnostic date to the date of the

induction failure, relapse, gave up, death caused by any reasons, or second tumor, whichever came fi rst, or the last contact with patients in continuous CR. Kaplan–Meier survival

curves were used to determine the diff erences in EFS between the patients with MICM and AFA classifi cation. Survival rates were calculated and compared using Kaplan–Meier

methods, log-rank tests, and Cox regression models (univariate and multivariate analyses). Due to the investigative nature of this analysis, all tests were conducted at the 1 %

signifi cance level. All analyses were performed using SPSS 16.0 (IBM, Armonk, NY, USA).

Results. We constructed a classier based on 240 published ALL microarray data and tested it on the 188 independent AFA samples. The accuracy of this cross platform prediction is

more than 94 % and the detail information of each subtype is shown below.

With the AFA-based gene expression profi ling classifi cation, 61.2 % (115/188) of patients were classifi ed as Standard risk group (SRG), 15.4 % (29/188) as High risk group (HRG)

and the others 23.4 % (44/188) as Intermediate risk group (IRG). The new classifi cation showed tiny disparity with the distribution of clinically assigned risk groups (the percentages

in clinical SRG, HRG and IRG were 71.3 %, 11.1 % and 17.6 %, respectively) (P > 0.05). The 5-year EFS for SR, IR and HR according to the new risk group stratifi cation were 94.3 ± 3.3 %,

67.1 ± 7.8 % and 48.3 ± 9.3 %, respectively, as compared to 92.9 ± 3.1 %, 66.3 ± 9.0 % and 28.6 ± 9.9 % (P > 0.05) according to the original clinical stratifi cation.

Conclusion. Despite AFA-based gene expression profi ling classifi cation with high accuracy, so far its prognostic value is not superior to the traditional MICM classifi cation. We will

enlarge the sample to validate its accuracy and follow-up all the patients to further observe its prognostic value.

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A B S T R A C T N O . : P - 3 0 9

Effi ciency of the ALL-MB-2008 protocol in children and adolescents

with acute lymphoblastic leukemia

A.I. Karachunskiy1, Yu.V. Rumyantseva1, O.V. Aleinikova2, O.I. Arakaev3, D.V. Litvinov1, A.F. Karelin1,

E.G. Boychenko4, S.N. Lagoyko1, O.I. Bydanov2, G. Henze5

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus;

3Pediatric Oncohematological Centre, Children’s Regional Clinical Hospital № 1, Yekaterinburg, Russia; 4City Clinical Hospital № 31 of Saint-Petersburg, Russia; 5Department of Pediatric Oncology/Hematology, Charité-Universitätsmedizin, Berlin, Germany

Key words: acute lymphoblastic leukemia, children, therapy, effi cacy

Introduction. Main characteristics of the ALL-MB (Moscow–Berlin) studies were the use of dexamethasone, prolonged asparaginase, a low cumulative dose of anthracyclines

and omission of high-dose chemotherapy. Here we present the results of the 3rd protocol generation – ALL-MB-2008, one aim of which was to evaluate whether the new risk

stratifi cation would improve event-free and overall survival (EFS, OS).

Aim. To evaluate the effi ciency of the original ALL-MB-2008 protocol within the multicenter study of acute lymphoblastic leukemia (ALL) treatment

Materials and methods. The randomized multicenter trial ALL-MB-2008 was conducted from February 2008 to January 2015. The database was frozen as of November 1, 2015.

Median follow-up was 3.12 years.

Results. 3598 patients eligible for analysis were recruited from 51 centers. According to the new criteria 1768 patients (49.1 %) were stratifi ed to SRG, 1521 patients (42.8 %) to ImRG,

and 309 patients (8.6 %) to HRG. For the total group of patients, the 8-year probability of EFS (pEFS) was 82.0 ± 1.0 %, pOS 86.0 ± 1.0 %; cumulative incidence (CI) of relapse was

11.0 ± 0.9 %, CI of isolated CNS relapse 1.0 ± 0.2 %. pEFS was 88.0 ± 1.0 %, 81.0 ± 1.0 % and 48.0 ± 3.0 % for SRG, ImRG and HRG patients, respectively. Outcome of SRG patients

with initial WBC ≥ 30 000/mm3 and spleen enlargement ≥ 4 cm from trial ALL-MB-2002 was compared with the respective patients of trial ALL-MB-2008 (now ImRG); pEFS was with

81.0 ± 2.0 % signifi cantly higher compared with 66.0 ± 2.0 % in the previous trial (P < 0.0001). Relapse rates were 6.3 % (ALL-MB-2008) and 19.8 % (ALL-MB-2002; P < 0.0001).

Conclusion. Overall results of ALL-MB-2008 study are signifi cantly better than ALL-MB-2002. The new stratifi cation system only based on clinical parameters as used in MB-2008 was

very successful and led to signifi cantly better overall EFS and reduced CI of relapses.

A B S T R A C T N O . : P - 3 1 1

The development of a medical device for L-asparaginase loading into red blood cells

D.V. Borsakova, E.I. Sinauridze, E.S. Protasov, F.I. Ataullakhanov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: red blood cells, acute lymphoblastic leukaemia, L-asparaginase, hypotonic dialysis

Introduction. L-asparaginase has been used as a part of therapy for acute lymphoblastic leukemia (ALL) in children and adults more than forty years. The enzyme catalyzes

the hydrolysis of asparagine to aspartic acid so that the blood levels of asparagine are reduced. It leads to dysfunction of a cell membrane and inhibition of nucleic acid and protein

synthesis. Healthy cells have an alternative mechanism of asparagine synthesis, which is catalyzed by asparagine synthetase. The activity of this enzyme is reduced or absent

in leukemic cells, thus a defi ciency of exogenous asparagine leads to its death.

At the same time L-asparaginase has a high immunogenicity and causes a wide range of side eff ects, including anaphylactic shock (an average of one third of patients), pancreatitis,

coagulopathy, immunosuppression, hepatic dysfunction.

In order to solve these problems new formulations that reduce the side eff ects of L-asparaginase and prolong its therapeutic eff ect were created. Presently L-asparaginase which is

bounded with a molecule of the polyethylene glycol is widely used. The PEG-L-asparaginase has an increased half-life (about 5–7 days) and immunogenicity that is signifi cantly lower

than the one of the native enzyme. There is still a need for new forms of L-asparaginase, which could improve the therapeutic eff ect and reduce the risk of side eff ects for the patient.

One of the most promising ways to overcome these diffi culties is the use of red blood cells as carries of L-asparaginase. The erythrocyte membrane prevents contact of the immune

system and the medicine circulating in the bloodstream.

Clinical studies have shown that the use of erythrocytes as carriers of L-asparaginase signifi cantly improves pharmacodynamic parameters of the medicine in comparison to the native

form (low levels of plasma L-asparagine last from 10 to 45 days), and reduces its immunogenicity (no allergic reactions in patients, C. Domenech, X. Thomas. Br J Haematol, 2011).

Aim. It is very diffi cult to use L-asparaginase loaded red blood cells in clinical practice. In order to resolve this problem we are developing the device that could automatically perform

a procedure of the L-asparaginase loading into erythrocytes.

Materials and methods. This device has both standard units such as a cells washing unit and a dialyzer and original units such as a controller of procedure parameters

and a disposable kit of plastic medical tubes and containers. The procedure for obtaining L-asparaginase loaded erythrocytes has four steps: separating red blood cells from plasma

and other undesirable elements and washing red blood cells, the enzyme incorporation into red blood cells by hypotonic dialysis, reasiling of cells’ pores arisen from the previous step

and fi nal washing.

Results. We constructed original units of device and defi ned the parameters for each step of the procedure. Now we are constructing the disposable kit and working under connection

all units together and coordination their work.

Conclusion. Red blood cells will fl ow through a closed circuit of a disposable kit. It will allow getting sterile and safe product, stable doses of the medicine, and give the opportunity

to work with small amounts of blood. As a result our device will allow using of L-asparaginase loaded red blood cells in the treatment of ALL in children.

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A B S T R A C T N O . : O P - 3 2 4

Outcome of Paediatric Philadelphia-Positive Acute Lymphoblastic Leukaemia (Ph+ve ALL)

Using Aggressive Chemotherapy with Imatinib in India

Brijesh Arora, Kalasekhar Vijayasekharan, Nirav Thackar, Gaurav Narula, Nikhil Patkar, Sumit Gujral, Prashant

Tembhare, Mani Subramaniam, Prathibha Amre Kadam, Shripad Banavali

Tata Memorial Hospital, Mumbai, India

Key words: Philadelphia-positive acute lymphoblastic leukemia, Imatinib, children

Introduction. Ph+ve ALL is a very high-risk subset of childhood ALL with historically poor outcomes without stem cell transplantation (SCT) before the advent and use of Imatinib

Mesylate. The incidence of Ph+ve ALL at our centre is higher at 7 % as compared to 2–3 % in the west. There is a paucity of data on the outcomes of Ph+ve ALL in India, where SCT

is not aff ordable for most patients.

Aim. We conducted a retrospective analysis of paediatric Ph+ve ALL patients treated with intensive chemotherapy with or without Imatinib.

Materials and methods. We audited records of Ph+ve ALL paediatric patients diagnosed between January 2005 – December 2014 who underwent treatment with institutional

ALL protocol (MCP-841) with or without Imatinib. No patient underwent SCT. EFS was calculated from date of diagnosis to date of relapse/progression while OS was calculated from

date of diagnosis to date of last follow up.

Results. A total of 104 patients were diagnosed with Ph+ ALL. The median age was 11 years (range 3–18 years). Male:Female ratio was 4:1. Median WBC count was 88,000 cells/mm3

(range, 870 – 642,000 cells/mm3). Nineteen patients had CNS involvement: 4 were CNS II (5 %) and 15 were CNS III (20 %). Of 94 patients who started therapy at our centre,

72 patients received Imatinib during their treatment: 29 during induction and 43 post-induction. Fourteen did not receive Imatinib and 8 abandoned therapy before response

evaluation. Median overall survival (OS) of the entire cohort was 18 months and estimated 3-year OS and EFS was 35 %. Overall survival for patients who received Imatinib at any

time during therapy was 41 %. However, none of the patients who did not get Imatinib survived for 3 years. Three-year EFS and OS in patients who received Imatinib in induction

was signifi cantly worse at 23 % compared to 49 % for those who started it post-induction (P = 0.03). However, there was no statistical diff erence in toxic deaths and morphologic

remissions between the groups.

Conclusion. Ph+ ALL is more common in India and presents with higher disease burden and CNS involvement. Outcome of Ph+ ALL without Imatinib and stem cell transplantation

is dismal. However, combined therapy including aggressive chemotherapy and Imatinib improves outcome.

A B S T R A C T N O . : O P - 3 2 6

Comparison Between UKALL and Lahore Protocol on Induction Response in Pre B,

Acute Lymphoblastic Lymphoma

Safi a Khan, Saadia Anwar

The Childrens Hospital & Institute of Child Health, Lahore, Pakistan

Key words: early response, remission

Introduction. Acute lymphoblastic leukemia (ALL) is most common childhood malignancy & Pre-B lymphoblastic leukemia is most common among acute leukemias. It is being

treated successfully with multiple anti-leukemic drugs. Diff erent regimens of chemotherapy are being used to treat leukemia with slight adjustment like COG ALL Protocols, UKALL

Protocol and Lahore Protocol. Lahore Protocol was designed couple of years ago, i.e. based on modifi ed BMF90 trail, to treat acute leukemia on internationally acceptable standards

that could be implemented in Pakistan. In all regimens, disease is stratifi ed as high risk, intermediate risk or standard risk before starting chemotherapy and then reassessed

for treatment response evaluation. In developing countries like Pakistan some tools of assessment like cytogentics, MRD (minimal residual disease) evaluation is not freely available

for risk group stratifi cation and treat response assessment.

Aim. To conduct a comparative analysis of induction response out come of Lahore Protocol and UKALL (Interim Guideline, 2011).

Materials and methods. Simple random sampling which is on of the non-probability sampling technique was used for sampling. Data of 50, Pre B Acute Lymphoblastic Leukemia

patients, up to 16 years of age, presented in Pediatric Oncology Department, The Children’s Hospital & Institute of Child Health, Lahore, Pakistan, in last 5 years, were reviewed

retrospectively for induction response out come. Half of the patients (n = 25) were treated on Lahore Protocol i.e. based on modifi ed BMF90 trail and remaining 25 patients got

induction chemotherapy as per UKALL interim guideline 2011. Both groups were compared in term of early response assessment and end of induction remission.

Results. Among patients who got chemotherapy as per Lahore protocol, 12 patients found slow responder in early response assessment whereas 20 were slow responder in UKALL

group. On comparison of end of induction remission, it is found that 22 patients out of 25 achieved remission while in other group 15 were able to achieve remission and rest 10 were

escalated for further chemotherapy at end of induction.

Conclusion. Based on data analysis, it is concluded that induction response of Lahore Protocol is better than UKALL due contextual arrangement.

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A B S T R A C T N O . : O P - 3 3 6

The impact of homocysteine level on methotrexate induced neurotoxicity in children

treated with acute lymphoblastic leukemia protocol

Mohamed Sedky Mahmoud Sedky1, Wael Khaled Zekri1, Mona Mohamed Aly Khalifa1, Sanaa Abd Elbaky Kenawy2

1National Research Centre; 2Faculty of Pharmacy, Cairo University

Key words: homocysteine, high dose methotrexate, neurotoxicity, acute lymphoblastic leukemia, lymphoblestic lymphoma

Introduction. Methotrexate (MTX) is an antimetabolite that is routinely used in the treatment of hematological malignancies and during its metabolism leads to hyperhomocysteinemia

that is associated with neurotoxicity.

Aim. The purpose of this prospective study is to determine whether the increase in plasma homocysteine (Hcy) concentration is related to MTX-induced neurotoxicity.

Materials and methods. We investigated these changes for both newly diagnosed Acute Lymphoblastic Leukemia (ALL) and lymphoblastic lymphoma (LL) pediatric patients

treated at the National Cancer Institute, Egypt. They were treated according to St. Jude total XV protocol to receive 2.5 or 5 g/m2 MTX as a phase of consolidation and were selected

between October 2009 and January 2010.

Results. Twenty nine patients were analyzed, M/F: 20/9, the mean age was 8 ± 4.4 years. Hcy level above 15μmol/L was considered positive. Hcy levels mean at diagnosis, pre 1st HD

MTX, post 1st HDMTX, Pre 2nd HDMTX, Post 2nd HDMTX were 12.10 μmol/L ± 4.17, 6.90 μmol/L ± 3.02, 17.59 μmol/L ± 6.00, 7.21 μmol/L ± 2.73 and 13.74 μmol/L ± 4.75 respectively.

Seventeen patients (58 %) had features suggestive of neurotoxicity. Positive Hcy levels were associated with neurotoxicity P 0.05, higher HDMTX 5 g/m2 P 0.023. A highly signifi cant

relation was found between initial Hcy level at diagnosis and fi nal Hcy level P 0.001; the same as between Hcy level Post 1st HDMTX and that Post 2nd HDMTX with P 0.006.

Conclusion. Plasma Hcy concentration was signifi cantly elevated after HDMTX administration and this elevation is associated with the observed neurotoxicity. Whether the elevation

in Hcy concentration can prove an informative biomarker for neurotoxicity requires additional testing with other MTX regimens.

A B S T R A C T N O . : O P - 3 5 0

Malignancies in infants: Epidemiologic profi le and outcome

Nabila Bouterfas, Ayda Mohand Oussaid, Zoulikha Zeroual, Yasmine Bouskia, Amghide Khati,

Mohamed Elmokhtar Khiari, Houria Boukhelal, Nafi ssa Keltoum Benhalla

CHU Beni Messous

Key words: infants, toddlers, neuroblastoma, nephroblastoma, leukemia, retinoblastoma

Introduction. Malignant neoplasms are relatively rare in infants, in this pediatric age group, cancer often have a diff erent clinical presentation from older children, supporting

the hypothesis of genetic predisposition. In addition, aim of management is challenging; getting remission with function organ preservation and reduced sequels.

Aim. The aim of this study is to determine cancer frequency, and to evaluate outcome of patients aged from 0 to 2 years old in our series.

Materials and methods. We retrospectively reviewed a series of 652 children diagnosed in the Pediatric Oncology Unit of CHU de Beni Messous, from 1st January 2005 to 31th

December 2015 as having a cancer. Patients were classifi ed and treated according to the conventional protocol regimens

All patients aged less than 2 years old were analyzed, with a follow up time varying from 1 to 120 months.

Results. Of 652 patients treated during this period, 236 infants were identifi ed, accounting for more than 36 %.There was no family history of cancer except in patients presenting

with bilateral retinoblastoma. Neuroblastomas represent the most frequent cancer with 55 %. Nephroblastomas and leukemias account for 14 %, followed by retinoblastoma,

teratoma, hepatoblastoma and rhabdomyosarcoma. Lymphomas were extremely rare, and reported in only 0.4 %.

30 patients presented with a bilateral solid tumor , including most frequently nephroblastoma(12 cases) and retinoblastoma (12 cases) than neuroblastoma (6 cases). 2 % of patients

have also developed a second malignant neoplasm, with a latency period ranging from 10 to 32 months. Overall survival rate was 60 %. Patients with neuroblastoma or leukemia

had the worst behavior outcome.

Conclusion. Diagnosis and management of cancer of children at this age constitute a real challenge. Even if prognosis remains favorable, sequelae should be considered in such

patients and require close follow-up.

A B S T R A C T N O . : P - 3 5 3

Relapses of acute lymphoblastic leukemia in children

E.A. Vaskina, E.V. Danilova, I.P. Tataurova, O.M. Tselousova

Kirov Research Institute of Hematology and Blood Transfusion, Russia

Key words: acute lymphoblastic leukemia, relapse, children

Introduction. The last decade is famous for improvement in outcomes of patients with ALL through the use of systemic chemotherapy and improved methods of diagnosis of this

disease, resulting in a 5-year free from treatment failure survival rate achieved – 75–80 % . However, 20–25 % of patients have got the relapses of the disease, which are the cause

of failure of treatment of children with ALLHA

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Aim. 225 patients with newly diagnosed ALL were monitored in the children’s hematology department of the Kirov Institute of Hematology in the period from 1991 to 2016.

Materials and methods. 40 (17.8 %) of children at diff erent stages of observation have relapses. The age of patients ranged from 2 till 18 years (median 7.0 years), the ratio of girls

and boys – 16/24.

Results. 17 (42.5 %) of the relapses classifi ed as very early, 8 (20 %) early and 15 (37.5 %) late. The structure of recurrences was as follows: isolated bone marrow relapse –

28 cases, the combined – 5, isolated extramedullar – 7. In the fi rst acute period 24 (60 %) patients were treated with protocol ALL-BFM-90, ALL-MB-2002 – 5 (12.5 %), ALL-MB-2008 –

6 (15 %) other protocols – 5 (12.5 %). Antireccurence therapy was conducted in 36 patients, the four did not receive treatment due to refusal of parents. 22 patients were treated

by ALL-REZ - BFM-90 protocol, 10 patients by protocol ALL-REZ -BFM-2002, and 13 American protocol – 3 patients, protocol Heltser – 1 patient. The second complete remission

(PR) was obtained in 55 % of cases (20 patients). Refractory to therapy was recorded in 6 (16.6 %), patients with early relapse. 10 (27 %) patients died of various complications

and progression of the disease before reaching remission. Of the 20 patients with the second PR 1 patient died of various complications during remission, 9 developed a second relapse

(3 patients are alive in one-third complete remission, and the third relapse was recorded in 1 patient, ended lethally). 12 (33 %) patients are in long remission.

Conclusion. 1. The frequency of relapse of ALL patients was 40 (17.8 %), and very early and late reccurence prevaled in relapse structure.

2. 12 (33 %) of patients are in complete remission.

3. The results were obtained in an incomplete analysis of risk factors, as well as immunophenotypic and cytogenetic studies were carried out in an incomplete volume, that is not

allowed to perform therapy in the fi rst acute period according to risk group.

A B S T R A C T N O . : P P - 3 5 7

Long-term outcomes of recombinant human erythropoietin therapy in anemic children

with acute lymphoblastic leukemia

V. Demikhov1, M. Lunyakova1, A.G. Beznoshchenko2, V. Skobin1, A.G. Rumyantsev3

1Ryazan Medical University named after I.P. Pavlov; 2Ryazan Regional Children’s Clinical Hospital named after N.V. Dmitrieva;3Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: anemia, pediatric ALL, recombinant erythropoietin, survival

Introduction. Anemia is common in children with acute lymphoblastic leukemia (ALL). The use of recombinant human erythropoietin (rHuEPO) is an alternative to blood transfusions.

High eff ectiveness of this option was demonstrated in adult patients with malignancies and in children with solid tumors. There are only several publications, which have reported

about effi ciency of erythropoiesis stimulating agents (ESA) in correction of anemia in ALL children, undergoing chemotherapy. However, there are some controversies in the impact

of the use ESA on the mortality or disease progression in adult cancer patients. So far there have been no evidence of a decrease in the survival among children treated with rHuEPO.

Aim. We analyzed effi ciency and long-term outcomes of rHuEPO therapy in ALL pediatric patients, undergoing chemotherapy by program ALL BFM-90m.

Materials and methods. Sixty two patients standard and intermedium risk group were enrolled in the clinical trial. The average age of the patients was 6.75 ± 0.93 years. Epoetin-

alfa was administered during an intensive phase of chemotherapy in doses of 200 IU/kg 3 times per week subcutaneously or 600 IU/kg once a week intravenously. All patients got iron

sulfate (II) in dose 5 mg/kg orally daily for a total period of ESA therapy. The average duration of ESA therapy was 24.5 ± 1.35 weeks (mediana – 27 weeks). In 14 (43.8 %) patients,

the ESA therapy has brooked by an average of 3.6 ± 0,36 weeks due to an increase in hemoglobin levels above 13.0 g/dl. Thirty children comparable by age and clinical manifestations

were control group (without ESA). The effi cacy of rHuEPO therapy was evaluated by an increase hemoglobin (Hb) levels, a reducing the number of transfusions and the volume of

transfused RBC concentrate. Long-term results were assessed by curves of the 5-year event-free survival (EFS), relapse-free survival (RFS) and overall survival (OS), constructed using

the Kaplan–Meier’s method.

Results. In the period of induction chemotherapy there was no diff erence in Hb levels and transfusion requirements between the ESA group and the control group. In the period of

consolidation chemotherapy ESA treated patients had higher Hb levels and needed less RBC transfusions. The average number of RBC transfusions was 4.7 ± 0.52 per patient in ESA

group and 6.7 ± 0.67 per patient in the control group (P < 0.05). The volume of the transfused RBC concentrate was 35.6 ± 3.37 ml/kg of the body weight in ESA group and 58.3 ± 6.44 ml/

kg in the control group (P < 0.05). The 5-year EFS for patients treated rHuEPO was 0.81 ± 0.04 compared to 0.73 ± 0.03 in control group, P = 0.51. The 5-year RFS was 0.93 ± 0.03

and 0.79 ± 0.04 in ESA group and control group respectively, P = 0.13. The 5-year OS for patients treated rHuEPO was 0.81 ± 0.04 compared to 0.73 ± 0.03 in control group, P = 0.35.

Conclusion. In our study the use of rHuEPO in children with ALL during chemotherapy according to the program ALL-BFM-90m leaded to signifi cant increase of Hb concentration

and reducing of the RBC transfusions. Five-year survival rate of patients with ALL treated rHuEPO did not diff er signifi cantly from those of the control group. There’s insuffi cient data

to conclude the lack of negative eff ect of ESA therapy on the survival of pediatric cancer patients. It’s necessary to carry out large multicenter, randomized, controlled trails to assess

long-term outcomes of ESA therapy in children with ALL.

A B S T R A C T N O . : O P - 3 6 1

Our Patients with ALL – Just a Phone Call Away

Rasel Siddique, Chowdhury Yakub Jamal, Md Anowarul Karim, Farzana Islam, Mehnaz Akter, Momena Begum,

Md Bani Yeamin, S M Rezanur Rahman, Lutfor Rahman, Md Tanvir Ahammed, Zannat Ara

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Key words: acute lymphoblastic leukaemia, a phone call away, our patients

Introduction. The most common childhood cancer treated at our centre is acute lymphoblastic leukaemia (ALL). To improve care, we initiated monthly meetings with parents to HA

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educate them on nutrition for children undergoing treatment, to identify the focus of infections, to explain complete blood count reports and to maintain communicate after discharge

from hospital.

Aim. To identify the focus of infections early over the telephone, to improve febrile neutropenia by starting fi rst dose of antibiotics and to reduce the cost of treatment.

Materials and methods. In March 2013, we initiated a 24 h telephone consultation service for parents of our patients. Queries from the parents and the provided solutions were

documented. The problems were solved by the doctor answering the phone or after discussion with others.

Results. From March 2013 to September 2015, we received 1000 telephone calls from 80 parents of current and new patients. Among the callers, 70 % had education level below

Class 10 (secondary school equivalent, around 15 years of age). Monthly income was between 125 and 375 USD. Thirty-fi ve percent of the calls were regarding medication: dosage,

continuing or stopping of chemotherapeutic drugs, or changes to drug schedule due to inconvenience or personal problems. The other 65 % were calls regarding infections, bleeding,

or queries on the CBC report. Infection-related problems diagnosed over the telephone included: respiratory tract infections, diarrhoea, UTI, perianal abscess, febrile neutropenia,

vomiting, headache, convulsion, and thrush. Solutions were provided accordingly. About 28 % of the cases resolved without need for more medical support. We tried to start the fi rst

dose of antibiotics over telephone, improving febrile neutropenia and reducing cost of treatment.

Conclusion. The 24 h telephone consultation service was welcomed by parents; we received an average of 1.1 calls a day. Most of the calls were regarding infections and related

problems. We were able to diagnose and resolve some of the problems over the telephone, negating the need for parents to make a trip to the hospital, helping to save costs.

A B S T R A C T N O . : P - 3 8 5

The experience of carrying out auto-HCT to the patient with Burkitt-type ALL

V.I. Nechaevskikh1, E.E. Zinina1, N.B. Popova1, S. Ponamorev2 1Surgut District Hospital, Russia; 2Ugra Research Institute of Technology, Russia

Key words: Burkitt-type ALL

Introduction. Introduction: In case of chemotherapy-sensitive relapse of lymphoma/leukosis of Burkitt’s type auto- or allo-HCT are prescribed. However, there is no accurate

recommendations concerning the treatment of such patients.

Aim. Objective: The clinical case of carrying out auto-HCT to the young patient in the second remission of a sharp lymph-based Burkitt-type leukosis is presented.

Materials and methods. Patient M., born in 1998, is diseased since October 2013, during medical examination concerning suspicion of cholecystitis in the city M., a diagnose

ALL on the bases of blastosis in peripheral blood, blastosis transformation of marrow (85.2 %), negative peroxidase reaction was diagnosed. The remission induction under

the Heltser’s protocol is carried out. In December 2013 the patient moved to the city of Surgut. The remission remained in the myelogram. Consolidation 1 under the protocol

ALL-MB-2008 began. On the score of course formations on the sternum, buttocks, chin, forearms appeared. The biopsy of the left forearm formation was carried out. Histologic

conclusion: Berkitt’s lymphoma. Further, reference medical histology medication was conducted in Federal Research Center of Pediatric Hematology, Oncology and Immunology named

after Dmitriy Rogachev, the diagnosis was confi rmed, t(8;14) and C-MYC gene transformation was indicated. Consequently, extra marrowy recurrence was stated in January, 2014.

6 blocks of programme В-NHL-M-2004 with Rituximab were conducted. The remission achieved, during PET pathological accumulation of medicinal radiocompounds was not

discovered. Taking into consideration the fact of related donor absence, the decision to carry out auto-HCT was taken. Stimulation G-CSF (fi lgrastimum) with apheresis CD34+ in amount

of 4.0 × 106/kg was made in July 2014. Ageing R-BEAM modifi cation Hovon from 22.07.14, auto-HCT 30.07–31.07.14. Complications: neutropenia 4th stage D+4, anemia 2nd stage

D+9, thrombocytopenia 4th stage D+8, febrile neutropenia. Grafting of auto transplant D+11.

Results. At planned CT-scan in September 2014, an uneven consolidation of cellular tissue, with sizes 36 × 34 × 45 mm was detected , results of PET-CT – metabolic active changes

in mediastinum (SUVmax до 8.5). Other signs of relapse were not presented. Consultation of leading experts of Federal scientifi c clinical center of children’s hematology named

after Dmitry Rogachev: radiotherapy, biopsy of formation are not needed. Dynamic supervision is continued. The last CT-scan in November, 2015: volume formation with the size

of 30 × 34 × 49 mm remains, accumulating non-active substance up to 68 HU, without signifi cant dynamics. Other changes were not revealed. The patient is examined regularly

by the hematologist, at present run no further signs of relapse appeared.

Conclusion. Carrying out auto-HCT to the patients with ALL Burkitt-type disease in the second remission can be seen as choice therapy in case of related donor absence.

A B S T R A C T N O . : O P - 3 8 6

Evaluation of risk factors and patterns of CNS complications in childhood haematological

malignancies during early phase of treatment

Zannat Ara, Chowdhury Yakub Jamal, Afi qul Islam, Md Anowarul Karim, ATM Atikur Rahman, Momena Begum,

Mehnaz Akter, Lutfor Rahman

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Key words: haematological malignancy, risk factors, CNS complications

Introduction. Central nervous system (CNS) complications during treatment of childhood. Haematological malignancies remain a challenging clinical problem. Although dramatic

improvement with risk stratifi ed chemotherapy and CNS directed therapy has signifi cantly increased the incidence and severity of neurotoxic events, number of published studies

is limited especially in children.

Aim. The purpose of this observational study is to evaluate the pattern of CNS complications along with related risk factors during early phase of treatment period in children with

haematological malignancies during early phase of treatment period in a single centre.

Materials and methods. This observational study was conducted in 50 children with hematological malignancies (ALL AML, NHL) were admitting in paediatric haematology

and oncology department of Bangabandhu Sheikh Mujib Medical University Dhaka, from july 2013 to june 2014. After clinical examination and positive lab investigation fi ndingsHA

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the diagnosed patients were treated according to specifi ed protocol based chemotherapy and was evaluated during early phase (induction phase) of treatment period.

Results. During one year period, total 12 patients out of 50 (24 %) showed CNS events. CNS complications included convulsion n = 9 (75 %), peripheral neuropathy n = 8 (66.6 %),

change in mental status n = 8 (66.6 %), altered conscious status n = 8 (66.6 %), L-asperginase related suspected complication n = 5 (41 %), IT therapy related toxicity n = 4 (33.2 % ),

cerebral infection n = 2 (16.6 %), cranial nerve involvement with unilateral hemiplegia n = 2 (16.6 %). Based on history, clinical lab fi nding diff erent possible risk factor or co-morbid

conditions for this events were expected like thrombocytopemia (75 %) with coagulation disorder, severe febrile neutroperia (66.6 %), H/O of exposure to traumatic IT therapy

3–4 days before CNS event (33.2 %) dyselectrolytaemia with hypocalcemia (16.6 %), H/O of ear infection (16.6 %), L-asparaginase related complication (41 %) and metabolic

abnormalities (8.3 %). Only 7 (58.3 %) of out of 12 patients were recovered from complications.

Conclusion. Central nervous system (CNS) complications during treatment of childhood haematological malignancies remain a challenging clinical problem This may be related

to various factors like inherent risk of disease itself , chemotherapy induced neurotoxicity and to some co morbid conditions along with the treatment method. As many of these

neurological complications are treatable, early diagnosis and prompt treatment is essential to limit permanent damage.

A B S T R A C T N O . : P - 3 9 5

Outcomes of childhood acute lymphoblastic leukaemia treatment according

to the ALL-MB-2008 and ALL-MB-2015 protocols in the Arkhangelsk region

N.A. Grigorieva1, A.S. Ulanova1, I.A. Turabov2

1Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsov, Russia; 2Northern State Medical University, Arkhangelsk, Russia

Key words: acute lymphoblastic leukaemia, children, morbidity, mean rate, event-free survival

Introduction. Hematoblastoses are the most common childhood cancer diseases, the major part of which is acute lymphoblastic leukaemia (ALL). ALL morbidity in the Arkhangelsk

region in 2008–2014 ranged from 1.04 to 4.99 per 100,000 of child population; mean morbidity rate for the given period is 2.75 ± 0.37. However, in the setting of scheduled therapy

optimization with strict adherence to the protocol procedures, a decrease in mortality rates and an increase in survival rates of patients with this disease are registered.

Aim. To evaluate the treatment outcomes of children with ALL who underwent therapy according to the ALL-MB-2008 protocol and some observations concerning the ALL-MB-2015

protocol.

Materials and methods. The analysis was carried out using the database of Medical Record Department of Arkhangelsk Regional Children’s Clinical Hospital (ARCCH) and Cancer

Registry of Arkhangelsk Regional Clinical Oncological Dispensary.

Results. The treatment of children with ALL according to various therapeutic regimens has been carried out at ARCCH since 1982. For comparison, overall survival (OS) in 1982–1991

was about 8.9 %. In 1991–2003 the ALL-BFM-90 protocol was applied; the survival rate increased up to 56 %. Starting from April 2003 therapy according to the ALL-MB-2002 protocol

was carried out, at this time, a 5-year survival rate amounted to 63.3 % (data as of early 2014). Later, in July 2008 the patients with ALL started to receive treatment according to

the ALL-MB-2008 protocol. A total of 39 patients aged 1.6–16 years old (median age – 3 years old) underwent treatment. The remission by Day 36 of therapy was achieved in 38 (97.4 %)

patients; 1 (2.5 %) patient died during the induction therapy. The risk group stratifi cation was as following: SRG – 17 (43.6 %), ImRG – 20 (51.3 %), HRG – 2 (5.1 %). Relapses were

registered in 4 (10.3 %) children; all of them turned out to be isolated bone marrow relapses, one of them was very early – in 1 (2.6 %) patient, early – in 1 (2.6 %) patient, late – in

2 (5.1 %) patients. Three patients (7.7 %) discontinued follow-up. In total 7 (17.9 %) patients died at the diff erent stages of therapy and in prolonged remission, 1 of which died

during the induction therapy, 2 – during consolidation therapy I, 1 – during consolidation therapy II and 1 - during consolidation therapy III, 2 patients died while being in relapse.

Hematopoietic stem cell transplantation was performed in 2 (5.1 %) children: one case of living-related transplantation (the patient died of complications); unrelated HSCT –

1 case (the patient is alive, in the second complete remission). To date, 29 children are alive, in remission (under follow-up). A 5-year overall survival rate (according to Kaplan-Meier)

amounted to 76.0 %; event-free survival rate – 71.3 %.The treatment of children with ALL according to the ALL-MB-2015 protocol has been introduced at our clinic since December

2014. In total 9 patients aged 3 - 14 years old were registered. Pre-B immunophenotype was found in most children: 2 patients – В2 and 6 patients – В2/В3, 1 child – Т2. Group

allocation was the following: group А – 3 children, group В – 4 children; group Т – ImRG – 1 patient; group D1 – 1 patient. Therapy allocation: induction – 1, consolidation I – 2;

consolidation II – 3; consolidation III – 1; under supportive therapy – 2. Eight children had achieved remission by Day 36, 1 patient is under induction therapy now.

Conclusion. Participation in a multicentre study and treatment according to the ALL-MB protocols resulted in signifi cant increase in the survival rate of patients with ALL. Treatment

optimization according to the ALL-MB-2008 protocol enabled us to achieve the reduction of mortality rate (induction mortality, fi rst of all) together with a good therapy response

(absence of non-responders), while the reduction of toxicity of scheduled therapy with the use of risk-adjusted schemes in the protocol version 2015 will make it possible to avoid

grave and fatal complications. Although event-free survival of the patients being under our supervision is far from perfect, a steadily growing trend toward better event-free survival

rates brings hope to further enhancement of treatment outcomes.

A B S T R A C T N O . : P - 3 9 6

Clinical signifi cance of genetic changes in childhood T-cell acute

lymphoblastic leukemia (ALL). Results of the multicenter group Moscow–Berlin (MB)

Yu.V. Olshanskaya1, A.N. Kazakova1, O.I. Soldatkina1, E.V. Aprelova1, G.A. Tsaur2, O.M. Plekhanova2, T.L. Gindina3,

D.V. Mercuriev4, L.V. Baidun5, S.N. Lagoyko1, Yu.V. Rumyantseva1, O.I. Bydanov1, G. Henze1, A.I. Karachunskiy1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Children’s Regional Clinical Hospital № 1 / Research Institute for Medical Cell Technologies, Yekaterinburg, Russia; 3Raisa

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D I S E A S E O R I E N T E DA B S T R AC TTO P I C

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Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia; 4Perm Regional Children's Clinical Hospital, Russia; 5Russian Children’s Clinical Hospital, Moscow, Russia

Key words: T-cell acute lymphoblastic leukemia, TLX3, TAL, gene rearrangement

Introduction. Published reports on the prognostic impact of genetic alterations in childhood T-cell ALL are controversial.

Aim. Aim of this study was to analyze whether results obtained in the Russian MB group are comparable with published reports.

Materials and methods. Patients were recruited during trial ALL-MB-2008 between February 2008 and January, 2015. T-cell ALL was diagnosed in 399 patients. Material for genetic

analyses was available in 120 patients. Besides routine karyotyping FiSH analyses were performed to reveal TLX3, TAL, TLX1, MLL and 9p rearrangements.

Results. The patient cohort comprised 30 girls and 90 boys; median age ranged from 1.2 to 17.5 years. According to cytogenetic and FiSH fi ndings, patients were grouped into: 1.

“Normal” – normal karyotype and FiSH results (n = 20); 2. TLX3 positive (n = 29); 3. TAL positive (n = 18); 4. TLX1 positive (n = 5); 5. MLL positive (n = 6); 6. 11p13-15 positive

(n = 5); 7. “Others” – any other cytogenetic aberrations detected by karyotyping or FiSH (n = 37).

According to the study regulations T-ALL patients were never standard risk. High-risk criteria for T-cell ALL was no remission on d36. In our series, 102 children (82.4 %) were allocated

to the intermediate and only 17.6 % to the high-risk group.

Probabilities of event-free survival (pEFS) and cumulative incidences of relapses (CI) for the major subgroups were: “Normal” 85 ± 8 % (CI 5 ± 5 %); TLX3 64 ± 10 % (CI 29 ± 10 %);

TAL 82 ± 9 % (CI 18 ± 10 %); “Others” 60 ± 9 % (CI 21 ± 8 %). Most favorable outcomes were observed for “Normal” and TAL. The highest CI of relapse was seen in TLX3; however

diff erences did not reach statistical signifi cance.

Conclusion. Patients with TAL rearrangement and those without any genetic abnormalities tended to have a better outcome, and the highest relapse rate was seen in TLX3 positive

patients. Insofar, our results are in line with others who could not clearly reveal signifi cant diff erences in outcome between the genetic subgroups. It is of note, however, that the

percentage of patients who had to be assigned to the high risk group was with only 17.6 % defi nitely lower than in other trials. The majority of patients in our cohort were by defi nition

intermediate risk and received only moderately intensive treatment in the respective arm of MB 2008. Nevertheless, overall pEFS was comparable with published results.

A B S T R A C T N O . : P P - 3 9 8

Endoplasmic reticulum stress-modulated miRNome in Jurkat cells:

integrative analysis of miRNA targets

D. Zaychenko, O. Lisina, M. Mesitov, R. Soldatov, A. Sokolovskaya, A. Moskovtsev

Institute of General Pathology and Pathophysiology, Moscow, Russia

Key words: T-cells, cell stress, microRNA, transcriptome

Introduction. A number of conditions interfere with oxidative protein folding processes in the endoplasmic reticulum (ER) can result in accumulation of misfolded proteins in the ER

lumen and inducea specifi c type of the stress responsereferred to as “ER stress”. Adaptation to ER stress depends on induction of the unfolded protein response (UPR), an intracellular

signaling pathways initiated by the activation of several sensors such as PERK, IRE1 and ATF6.

Aim. Stress is accompanied by changes in the RNA turnover, in particular, activation of multiple mRNA degradation pathways, IRE1 -mediated miRNAdecay. UPR activation leads to

signifi cant upregulation of genes involved in maintaining homeostasis of the ER and protein folding, including chaperones and foldases.The aim of our study was to clarify the eff ect

of ER stress on cellular miRNome, and the role of microRNAs in the posttranscriptional regulation of ER-stress response genes that are poorly understood.

Materials and methods. To identify diff erentially expressed miRNAs under ER-stress Next-Generation Sequencing (Illumina platform)of small RNA was performed from Jurkat cells

(human T lymphocytes, acute T cell leukemia) treated with 2,5mM dithiothreitol(6 hours)to induce ER-stress.BiP, DDIT3 and XBP1 mRNA levels were analyzed by Real-Time PCR. Cell

death was measured after 24 hours treatment by fl ow cytometry.Also we used genome-wide gene expression analysis using Aff ymetrix Gene Chip Gene 1.0 ST Array System with

subsequent bionformatic annotation and search of microRNA targets

Results. The expression of 49 miRNA was found to be signifi cantly diff erentially regulated in ER-stressed cells versus controls. Of these, 20 miRNAs were upregulated while

29 miRNAs were downregulated. In order to characterize the functional signifi cance of these diff erently expressed microRNA, we performed genome-wide gene expression analysis

using Aff ymetrix Gene Chip Gene 1.0 ST Array System with subsequent bionformatic annotation and search of microRNA targets. 1480 genes were found to be diff erentially expressed

in response to ER-stress with FDR < 0.05. Of these, 1019 genes were upregulated and 461 genes were downregulated. Functional annotation of diff erentially expressed mRNAs

was carried out using Gene Set Enrichment Analysis (GSEA). In agreement with general expectations, the top processes enriched with the upregulated genes involve UPR signaling

(activation of chaperone genes by XBP1S, activation of genes by ATF4, PERK regulated gene expression, cytosolic tRNA aminoacylation, amino-acid metabolism and immunity (amino

acid synthesis and interconversion transamination, amyloids, antigen presentation folding assembly and peptide loading of class MNC (major histocompatibility complex), systemic

lupus erythematosus), diabetes pathways, positive regulation of RNA polymerase I and III transcription (RNA Pol I promoter opening, RNA Pol I transcription, RNA Pol I and RNA

Pol III and mitochondrial transcription), nuclear homeostasis (packaging of telomere ends, telomere maintenance, deposition of new containing nucleosomes at the centromere,

chromosome maintenance). The most down-regulated processes during ER-stress were mRNA splicing, processing of capped intron containing pre-mRNA, taste transduction, mRNA

processing.

Conclusion. We found weak positive correlation between mRNA and miRNA expression that could point to dysregulation of miRNA machinery at this stage of ER stress.

This dysregulation might be a prominent feature of cancer cells.

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A B S T R A C T N O . : P - 1 0 0

Long-term follow-up of children with chronic myeloid leukemia after tyrosine kinase

therapy at the Republic of Bashkortostan

E.V. Yakupova, T.N. Krasavtceva, E.F. Amirova, A.I. Makhonina, V.B. Makhonin, R.N. Stepanova

Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan

Key words: chronic myeloid leukemia, tyrosine kinase inhibitor, children

Introduction. Tyrosine kinase inhibitor (TKI) have become the standard fi rst-line treatment for chronic phase of chronic myeloid leukemia (CP-CML) and transformed CML from fatal

disease into a leukemia subtype with a favorable prognosis.

Aim. The purpose of this study is to report long-term outcomes of CP-CML patients at the Republic of Bashkortostan (RB).

Materials and methods. This is a retrospective analysis from the CML patients case records from RB. The method of limiting dilution was used to assay of BCR-ABL1 transcript level

for the monitoring of the response to therapy (MRD).

A total of 8 CP-CML patients less than 14 years old at the time of diagnosis (with a median age of 10 years 1 month) registered in RB from a period of 2002 to 2015 year were enrolled

in this study.

Results. According to our research the annual incidence of CML in children at the RB is 0.08/100 000. Now we have 4 pediatric patients with CP-CML under the observation. Patient 1. Major molecular response (MMR) was received after 4 months treating with 400 mg imatinib mesylate (IM), after 14 months was diagnosed molecular response (MR) and it is saving

during 4 years. Patient 2. MMR (10-4) was received after 3 months treating with 400 mg IM, after 10 months’ therapy BCR-ABL expression level 0.014 %. Patient 3. MR was received

after 3 months treating with 400 mg IM and it saved during 3.5 years, but in the last test was determined slight increase BCR-ABL expression level (10-4). Patient 4. MR was received

after 12 months treating with 400 mg IM and it saved during 2 years, but late was determine increase BCR-ABL expression level (10-2) and IM dose was escalated to 600 mg, after

18 months’ treatment there is decrease in the expression level for 3 log (10-5).

Two patients 24 and 26 years old are taking IM 400 mg during 10 and 12 years respectively, at this period of time they saved complete hematological response (CHR) and MMR. Patient

R. MMR was received after 4 months treating with 400 mg IM and it saved during 4 years, but late was determine increase BCR-ABL expression level (10-4) and IM dose was escalated

to 600 mg, after 12 months’ treatment IM 600 mg loss of cytogenetic response was registered and IM dose was escalated to 800 mg, during 3 years’ treatment IM 800 mg MR was not

still received and treatment was transitioned to the second-line ITK dasatinib 100 mg. At the dasatinib MR was received and it is saving during 4 years. Patient A. MMR was received

after 16 months treating with 400 mg IM but due to irregular IM taking was diagnosed blast transformation and death from progression of disease.

Conclusion. All the children were started on IM at a dose 400 mg. CHR was obtained within two months and confi rmed thereafter monthly. 7 patients are alive. Hyperpigmentation,

edema, growth retardation, nausea is the most common manifestations of adverse events (AE). AE were tolerable and did not require discontinuation of TKI therapy. IM dose escalation

or switching to the second-line ITK dasatinib improve the results of therapy. Compliance between the patient, physician and close relatives increase the eff ectiveness of therapy.

Acknowledgment. Authors thanks the laboratory of biological microchips for MRD testing.

MYELOID LEUKEMIAS, MYELODYSPLASTIC AND MYELOPROLIFERATIVE SYNDROMES

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A B S T R A C T N O . : O - 1 0 8

Hematopoietic stem cell transplantation signifi cantly improve outcome of infants

with acute myeloid leukemia carrying translocation t(7;12)(q36;p13)

G.A. Tsaur1, E.A. Zerkalenkova2, O.M. Plekhanova1, E.V. Fleischman3, E.V. Volochnik4, I.I. Kalinina2, O.I. Sokova3,

Yu.V. Olshanskaya2, A.M. Popov2, O.R. Arakaev1, L.I. Saveliev1, E.V. Shorikov1, L.G. Fechina1

1Children’s Regional Clinical Hospital № 1/Research Institute for Medical Cell Technologies, Yekaterinburg, Russia;2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

3N.N. Blokhin Russian Cancer Research Center, Moscow, Russia;4National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: acute myeloid leukemia, infants, treatment outcome, t(7;12)(q36;p13)

Introduction. Translocation t(7;12)(q36;p13) leading to MNX1 (HLXB9)-ETV6 fusion gene formation is recurrent chromosomal rearrangement, predominantly found in children

under 24 months of age with AML and associated with dismal outcome. In the majority of cases t(7;12) is cryptic and can be detected by fl uorescent in situ hybridization (FISH) only.

Aim. To evaluate initial clinical parameters and input of hematopoietic stem cell transplantation in infants with AML carrying t(7;12) using novel 3-color FISH approach.

Materials and methods. We retrospectively analyzed data of 81 infant AML cases, diagnosed between June 1999 and December 2014. Median age was 6.9 months (range 0.3–11.7).

FAB variant was available in 72 patients. Chromosomal banding analysis (CBA) was performed in all cases, FISH for MLL rearrangements was done in 49 cases. All MLL-negative cases

were screened for t(7;12) by novel 3-color FISH approach consisted of a break-apart probe composed of an orange labeled probe centromeric to the ETV6 gene in 12p13, a green

labeled probe telomeric to ETV6 together with a probe combination made of two loci fl anking the HLXB9 gene, both labeled in blue (MetaSystems, Germany).

Results. Median age of t(7;12)-positive patients was similar to t(7;12)-negative ones (7.0 vs 6.9 mo, P = 0.241). All 6 patients with t(7;12) referred to AML high-risk group. Median

WBC count was 29.7 × 109/L (range 15.7–210). Five of 6 patients had initial CNS disease, in all cases extramedullary organ involvement was observed. Three out of 6 t(7;12)-positive

patients had immature FAB variants (one AML M0 and 2 AML M1), 2 patients had AML M5a and 1 biphenotypic acute leukaemia. Translocation t(7;12) was associated with immature

immunophenotype: CD34-positive and CD117-positive cells were revealed in all 6 cases, that was signifi cantly more common in comparison to t(7;12)-negative cases (P = 0.004

and P = 0.038, respectively). Interestingly, co-expression of CD7 antigen was detected in 5 out of 6 t(7;12)-positive cases and this phenomenon was observed in t(7;12)-positive

group more frequent than in t(7;12)-negative ones (P = 0.001). In fi ve out of 6 cases t(7;12) was cryptic and was not found by CBA. Hematopoietic stem cell transplantation (HSCT)

was performed in the 1st remission in 4 cases (including 2 autologous HSCT) and in the 2nd remission in 1 case. EFS and OS were similar in t(7;12)-positive and t(7;12)-negative groups:

EFS (0.33 ± 0.16 vs 0.39 ± 0.07, P = 0.573 and 0.60 ± 0.21 vs 0.45 ± 0.08, P = 0.571, respectively) with median of follow-up time 36 months.

Conclusion. Application of HSCT in both 1st and 2nd remission could overcome unfavorable infl uence of translocation t(7;12)(q36;p13) and led to OS equal to general cohort of infant

AML. Among MLL-negative patients t(7;12) was the most common chromosomal aberration detected in 19.4 % of cases.

A B S T R A C T N O . : P P - 1 1 3

Outcome of the pediatric patients with normal karyotype acute myeloid leukemia

in Yeungnam region, South Korea: multi-center retrospective analysis

Chueh Hee Won1, Lim Young Tak2, Kim Heung Sik3, Lee Jae Min4, Hah Jeong Ok5, Lee Kun Soo6, Park Jeong A7,

Park Jikyoung7, Lim Jae Young8, Kim Ji Yoon6, Lee Mi Ji1

1Dong-A University Hospital; 2Busan National University; 3Keimyung University; 4Yeungnam University; 5Fatima Hospital; 6Kyungpook National University; 7Inje University Busan Paik Hospital; 8Gyeongsang National University

Key words: normal karyotype AML, HSCT

Introduction. Recent progress on genetics, many karyotypes and genetic information which can refer prognosis, and furthermore, guiding the future treatment, have been found in

acute myeloid leukemia (AML). However, normal karyotype AML, which consist almost half of the AML, doesn’t have any conclusions about its prognosis.

Aim. Therefore, in this study, outcome of the patients with normal karyotype AML in Yeungnam region of South Korea to make the guide for future treatment.

Materials and methods. Medical information of the patient who diagnosed and treated in Yeungnam region hospital between 1st Jan 2000 and 1st Aug 2015 were collected and

analyzed retrospectively.

Results. Total of 235 patients were diagnosed as AML in Yeungnam region, and the 197 patients received treatment at the hospital located in Yeungnam region. Among them,

36 patients were normal karyotype AML. 8 patients showed CR after 1st induction chemotherapy, but 26 patients achieved CR after all induction phase. Among the patient who achieved

CR, 5 patients relapsed, and only 1 patient alieved. 14 patients received allogeneic hematopoietic stem cell transplantation (HSCT), and rest of them received only chemotherapy.

7 patients who received only chemotherapy were alive. The overall survival rate was 63.8 %, and the median survival time was 7.5 years. The overall survival of the patients who

received allogeneic HSCT was 78.6 %, and the median survival time was 8.5 years. The survivals of the patients who received allogeneic HSCT superior signifi cantly compared with

that of the patients who did not received HSCT.

Conclusion. Patients with normal karyotype AML showed relatively good outcome. The survival rate of the patients who received allogeneic stem cell transplantation was signifi cantly

higher than that of the patients who did not. But due to the short-hand of this study, the roll of HSCT should be evaluated through further study.

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A B S T R A C T N O . : O P - 1 2 1

The features of immunological and morphocytochemical diagnosis of acute

megakaryoblastic leukemia

O.I. Illarionova1, S.A. Plyasunova1, M.E. Dubrovina1, T.V. Konyukhova1, E.Y. Osipova1, E.B. Rusanova2, E.E. Zueva2 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2First Pavlov State Medical University of St.Petersburg, Saint Petersburg, Russia

Key words: fl ow cytometry, immunophenotyping, acute leukemia, megakaryoblastic leukemia, megakaryoblasts, laboratory diagnosis

Introduction. Acute megakaryoblastic leukemia is a rare pathology that amounts up to 0.5 % of all acute nonlymphoblastic leukemias, including 1–10 % acute lymphoblastic

leukemias (AML) in adults, 3–10 % childhood AML and 20 % AML in newborns. The incidence of AML-M7-variant in children with Down syndrome is 500 times higher than

the incidence of children without this pathology.

Aim. To analyze the features of immunological and morphocytochemical diagnosis of acute megakaryoblastic leukemia.

Materials and methods. Over the period from 2012 to 2015 “acute megakaryoblastic leukemia” was diagnosed in 22 children (15 boys and 7 girls) based on the analysis

of cytometry data of 2731 patients. The biological material for diagnostic investigation was presented as a bone marrow. Probe preparation was performed on a staining-lysis-

washout technique. The fl ow cytometry was performed on BD FACSCanto II cytometer. The analysis of the fl ow cytometry data was carried out with BD FACSDiva Software.

Results. The diagnosis was stated when detecting not less than 20 % of blasts in the bone marrow and at least the half of them had megakaryocytic diff erentiation indications.

In a low level of diff erentiation megakaryoblasts are similar to myeloblasts and lymphoblasts, blasts with “lymphoid” morphology and cytoplasm budding; in a more diff erentiated

forms megakaryoblasts are of medium (12–18 mсm) and large size with less diff erentiated cells with high nuclear-cytoplasmatic relation. Nuclei of megakaryoblasts are round and

slightly irregular-shaped or with impressions with a delicate chromatin network, with 1–3 nucleoli. A homogeneous distribution of chromatin and hyperchromia of nuclei are typical

characteristics; cytoplasm of cells is basophil, frequently creates evagination or pseudopods; Auer bodies were not observed.

Cytochemical assessment of AML-M7 blasts: myeloperoxidase, chloroacetate esterase and lipids were absent. Immunophenotyping allows to state with accuracy the megakaryostic direction

of blasts diff erentiation, to carry out the diff erential diagnosis with such pathologies as acute lymphoblastic leukemia, other forms of myeloblastic leukemias, metastases of malignant tumors

to bone marrow. Immunologically in M7-variant expression of one or more platelet antigenes were detected: СD41а (glycoprotein IIb), CD61 (glycoprotein IIIа), as well as CD42b (glycoprotein Ib),

that is common in more mature cells. CD36 antigene expression is a typical characteristic. In the majority of cases the expression of CD13 and CD33 myeloid antigenes, CD7, CD4 T-cell antigenes,

CD2 (rarely), NK-associated CD56 was detected on blastic cells. Also in the range of cases there were lineage-nonspecifi c CD34, CD38, CD117 antigenes on blastic cells. B-cell markers were negative.

Conclusion. The assessment of megakaryocytic markers requires caution as in many pathological states platelets stick to larger cells, monocytes and neutrophils, and as a result they

create positivity eff ect on markers of megakaryocytic direction diff erentiation. Therefore when planning the assessment of megakaryocytic markers it is necessary to wash the sample

in advance in order to remove adherent platelets. Considering the possibility of platelet satellitesm and clinical signifi cance of diagnosis it is not recommended to assess samples that

are not fresh; samples received with non-implementation of shipment regulations; samples received after the start of the treatment.

The features of AML-M7 blasts allow to make an immunophenotupic diagnosis during 1 business day after the receipt of biological material.

A B S T R A C T N O . : O P - 1 9 7

Morphological characterization of the leukocytes bound to an anti-CD antibody

microarray allows suggesting preliminary diagnosis in acute myeloid leukemia

S.A. Kuznetsova1, A.N. Khvastunova1, A.O. Zakirova1, O.S. Fedyanina2, A.A. Maschan1, F.I. Ataullakhanov1 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Center for Theoretical Problems of Physicochemical Pharmacology, Moscow, Russia

Key words: morphology, cluster of diff erentiation, cytochemistry, microarray

Introduction. The diagnosis of acute leukemia and related conditions starts from the analysis of morphology, cytochemistry and immunophenotyping of the bone marrow aspirate.

In case of acute myeloid leukemia (AML) morphology and cytochemistry are crucial for the diagnosis while the immunophenotyping confi rms the myeloid origin of the blasts but is

not suffi cient to distinguish between the AML subvariants. A technique permitting to determine the immunophenotype of a leukocyte with certain morphology would facilitate the

comparison of the two diagnostic methods and would increase the reliability of the diagnosis.

Aim. We have earlier suggested an anti-cluster-of-diff erentiation (anti-CD) antibody microarray on a transparent support for leukocyte sorting and a method for preparation of the

microarray-bound cells for high-resolution morphology analysis. This work aimed to develop a microarray-based method for preliminary diagnosis of AML.

Materials and methods. The suspension of mononuclear cells separated by density gradient from bone marrow aspirate is incubated with the microarray in non-mixing

conditions. After the unbound cells are washed away the leukocytes on the microarray are grouped according to their surface CD antigens. The microarray-bound cells are then dried

and fl attened using a home-developed cytocentrifuge resulting in a “cell-sorted” smear. We have studied the bone marrow aspirates of 40 pediatric patients with AML (М5 – 10 patients,

M4 – 11 patients, M7 – 6 patients, M3 – 5 patients, M2 – 5 patients, M1 – 3 patients) and 20 patients with acute B-lymphoblastic leukemia (B-ALL).

Results. We have shown that morphological and cytochemical characteristics of the microarray-bound cells are close to their characteristics in smears. We suggest a diagnostic

strategy for diff erentiation between AML and ALL based on morphological blast detection and determination of blast immunophenotype by analyzing their relative amount

among the cells captured by the antibodies against all the CD antigens. We also show that the further diff erentiation between the AML subtypes can be done using the leukocyte

cytochemistry (myeloperoxidase and nonspecifi c esterase).

Conclusion. Combined analysis of the pathologic cells’ immunophenotype, morphology and cytochemistry on the microarray permits to arrive at preliminary diagnosis and can be

used in cases of any controversies between morphology, cytochemistry and immunophenotyping. This work is partially supported by RFBR grants 16-34-01030 and 16-04-00282.

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A B S T R A C T N O . : P - 2 1 5

Case of successful treatment of acute myeloid leukaemia with extramedular aff ection

at child in Tyumen region

A.Yu. Chernova, T.I. Ksenzova, L.A. Ilarionova, E.V. Anikina, O.V. Ananieva, O.V. Sofeicova

Regional Clinical Hospital № 1, Tyumen, Russia

Key words: myeloid leukaemia, clinical case, extramedullar aff ection

Introduction. Literature data shows that acute lymphoblastic leukaemias (ALL) occupies about 80 % of cases among oncological disease at children and acute myeloid leukaemias

(AML) – 15–17 %. Extramedular aff ections (EA) in case of AML diagnosed in 3 % of cases. Ten – twenty cases of acute leukaemias diagnosed every year in Tyumen region at children,

AML occupies 10–45 % among them. Twenty six cases of AML at children were revealed in Tyumen region, in 4 (15 %) cases EA was diagnosed.

Aim. To show the features of diagnosing and treatment of AML with EA on the clinical case demonstration.

Materials and methods. Boy M., 14 years old, during one month suff ered from pain in chest, cough, dyspnea. Entered the department of haematology and chemotherapy

of Regional clinical hospital No. 1 (Tyumen) in the very severe condition. Defense attitude, fever to 38.0 °С. Skin surface pale-grey, weeping, lymph nodes conglomerates with

diameter to 2 sm. All abdominal cavity was occupied by palpated volume mass. CT scan of mediastinum showed: nodular lesions located paratracheally up to 6.0 sm. in diameter

with confl uent characteristics. These lesions sharply attached inner vessels, hydrothorax at right with prolongation to interlobar pleura and compression of lung parenchyma on 1/3,

hydropericardium. Lesion of soft tissue density cavity was registered in parapancreatical area with expansion to the area of portal fi ssure and in para-aortal area, to the small pelvis

with compression of pancreas, all vessels of this area, bile ducts, nodules lesions under the diaphragm, on peritoneum longitude, in small pelvis to 5.3 sm., perineal lymph nodes up

to 2.0 sm. in diameter, hepatosplenomegaly, ascite. Toracocentesis were done multiple times, 450 ml to 1500 ml of fl uid were evacuated. Bone marrow aspiration – blast cells – 71.8 %,

myeloperoxidase activity – 2 %, glycogen in cells – 80 % in diff use and diff use-granular form, lipids in cells – 100 % negative, activity of non-specifi c esterase – 90 %, full blocked by

sodium fl uoride. Cells of bone marrow region (CD45+) 38 % with the following phenotype: HLA-DR+ CD34- CD38+/- CD58+ CD117- CD33+ CD4+ CD64+ CD36+ CD11b-/+ CD11c-/+ CD15+

CD65+ CD56+ MPO+ Ki67+ med BCL2+ CD19- CD10- CD22- CD79a- CD13- TdT- CD20- s cyt K/L/IgM- NG2-/+s cytCD3-, what is characterize AML, M5 is possible. Myelosarcoma with EA

verifi ed: volume lesion in abdominal cavity, retroperitoneal cavity, small pelvis, lesion in chest, specifi c pleurisy, ascite, pericarditis, bone marrow involvement. Histological verifi cation

of EA, cytogenetic study was not done due to bad status of patient. Two courses of induction 7+3 were done (rubomycin 60 mg/sq.m.), 3 courses of HD Ara-C 1 gr/sq.m., IdaAC-Ida,

IdaAC-Mito. Specifi c therapy was complicated by myelotoxic agranulocytosis, double-sided pneumonia, mucosytis, gastroenterpathy, invasive hepatolyenal mycosis after third course

of consolidation. Multiple hypodensive lesions in liver sized 5–10 mm with fuzzy contour were revealed. Successfully treated with voriconazole.

Results. Complete bone marrow remission and reduction of EA was reached based on the therapy. Match related bone marrow transplantation in Saint-Petersburg Raisa Gorbacheva

Memorial Research Institute of Children Oncology, Hematology and Transplantation was done. Follow-up continuing.

Conclusion. EA at AML at children is predictors of severe clinical condition and unfavorable prognosis. Remission can be reached on background of program high-dose chemotherapy

and bone marrow transplantation.

A B S T R A C T N O . : P - 2 3 1

Myasthenia gravis and chronic myeloid leukemia in a child

N. Tursunova, I. Erimbetova, Kh. Khazakbaeva, G. Ismatova

Scientifi c-Research Institute of Hematology and Blood Transfusion, Tashkent, Republic of Uzbekistan

Key words: myasthenia gravis, chronic myeloid leukemia, child, therapy

Introduction. Myasthenia gravis is an autoimmune disease, with transient muscle weakness and abnormal fatigue. The prevalence of myasthenia gravis is from 1 to 5 per 100 000

population. Children and adolescents up to 17 years make up 9–15 % of patients with myasthenia gravis. Usually disease begins from 7.2 years (in average). The annual frequency

of CML is 1–1.5 per 100 000 population. Chronic myeloid leukemia is very rare among children, no more than 1–2 % of childhood leukemia. Nowadays 80 children with CML receive

therapy with tyrosine kinase inhibitors of the 1st generation in Uzbekistan.

Aim. To study the characteristics of chronic myeloid leukemia and myasthenia gravis in children.

Materials and methods. Boy (11 years old) was ill from the spring of 2014, weakness, lethargy, decreased motor activity, and ptosis were observed. CBC: Hb 101g/L, white

blood cells 83.3 thousand, a shift to the left to metamyelocytes 10 %. RT-PCR BCR-ABL p210-200, 89 %. Chronic myeloid leukemia was diagnosed, with following prescription

of gidroksikarbomid 2000-1000 mg, and further imatinib 340 mg/m2. The dynamics of the RT PCR M-BCR-ABL p210 –61.42 %. In a year after diagnosing CML problems with chewing

and swallowing food, active and passive movements were limited, nasal voice, then joined paresthesia, orthostatic overload intolerance were periodically detected. Muscle tone was

lowered. Abdominal refl exes were weak. Neurologists examined the patient, after electromyographic study was provided they diagnosed myasthenia gravis. Symptomatic treatment

with anticholinesterase drugs, small doses of corticosteroids and therapeutic plasmapheresis were prescribed and executed.

Results. During imatinib monotherapy, clinical-hematological improvement and absence of cytogenetic and molecular responses were noted. The myasthenia gravis aggravated the

patient’s condition. Combined therapy of both diseases did not lead to a positive result. The patient died in six months after diagnosis of myasthenia gravis.

Conclusion. Chronic myelogenous leukemia is rare among children and teenagers. A myasthenia often appears among children. The combined therapy of two competing diseases

failed to stabilize the situation and led to patient’s death. It is necessary to develop the principle of CML therapy in combination with autoimmune diseases, such as myasthenia gravis.

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A B S T R A C T N O . : P - 2 3 2

Myeloproliferative disorders and leukemia in newborns with Down syndrome

T.V. Stepanova1, G.V. Trubnikova2, M.V. Budanova1, A.F. Karelin2

1Voronezh State Medical University named after N.N. Burdenko, Russia; 2Voronezh Regional Children’s Clinical Hospital № 1, Russia

Key words: Down syndrome, newborn, leukemia

Introduction. The type of congenital leukemia must be distinguished from leukemic reactions and Down transient myeloproliferative disorder. Children with Down Syndrome (DS)

have a markedly enhanced incidence of myeloid (ML-DS) and lymphoid (DS-ALL) leukemias. The risk of DS-ALL has been estimated to be 10–20 times higher than sporadic ALL1-2.

In most published multi-institutional ALL protocols DS-ALL comprises about 1–3 % of total patients. This suggests a leukemogenic role of constitutional trisomy 21. The role of the

trisomy 21 has remained a mystery.

Aim. We present two cases of Down transient myeloproliferative disorder and congenital leukemia in newborns.

Materials and methods. Transient myeloproliferative disorder is a rare condition in the neonatal period, connected with trisomy, or other abnormalities of chromosome 21.

It is characterized by high blastosis in peripheral blood and bone marrow and it usually resolves without specifi c therapy in 1 to 3 months.

The fi rst patient was a boy with Down syndrome, karyotype – 47XY+21 and a cardiac defect (common atrioventricular channel anomaly). His mother was 39 years old,

had professional diseases. The second patient was a neonate female with Down syndrome. Her mother was a healthy woman 21 years old,

Results. This male neonate manifested with hyperleukocytosis (85.0 × 109/l), blastosis (79 %) in peripheral blood and bone marrow (59 %) detected at 6 days of age. His liver and

spleen were enlarged. No specifi c antileukemic therapy was given. After 3 weeks leukocytosis and blastosis were decreased, and we observed normalization of peripheral blood.

At 3 months of age the bone marrow smear was normal.

The girl showed leukocytosis (57 × 109/l) and blastosis (46 %) in peripheral blood and bone marrow (69 %) at age of 12 days. In the second case congenital myelomonoblastic

leukaemia (M4 in FAB classifi cation) was diagnosed. She was treated according to BFM for acute myeloblastic leukemia protocol and maintained a complete remission. Recurrence

of leukemia occurred after 11 months. The second remission was remained during of 6 months. Finally the girl died of progressive congenital leukemia after 18 months.

Conclusion. Despite of the great progress in the treatment of childhood leukemia, prognosis of congenital leukemia is sad. These cases confi rm the diffi culties in diff erentiation

between congenital Down leukemia and transient myeloproliferative disorder.

A B S T R A C T N O . : O P - 2 3 9

Eff ectiveness, toxicity and place of epigenetic therapy

in childhood acute myeloid leukemia

A.V. Popa, V.S. Nemirovchenko, G.L. Mentkevich

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: acute myeloid leukemia, epigenetic therapy

Introduction. The results of treatment children with acute myeloid leukemia (AML) are not satisfi ed yet. The standard chemotherapy allows achieve complete remission in 92–96 %

of patients, but disease free survival (DFS) and event free survival (EFS) are not good yet. Many AML study groups could decrease count of some complications which developed during

chemotherapy, but not avoided of relapses which developed from residual leukemia stem cell.

Certain cytogenetic abnormalities and gene mutations are well-known and have been recognized in the World Health Organization classifi cation. AML results from collaborating

genetic aberrations in at least 2 diff erent classes. Type I aberrations induce uncontrolled proliferation and/or survival of the leukemic cell and are often represented by activating

mutations in genes involved in signal transduction pathways, such as FLT3, KIT, NRAS, KRAS and PTPN11. Type II aberrations inhibit cell diff erentiation and mainly result from

genetic aberrations in hematopoietic transcription factors, due to, for instance, AML-characteristic translocations t(8;21)(q22;q22)/AML1-ETO and 11q23/MLL rearrangements,

or from mutations in NPM1 and CEBPA genes. However, certain aberrations found in AML, including those related to epigenetic deregulation, do not completely fi t into the current type

I and type II classifi cation. Research into the epigenetic control of gene expression in AML, including histone modifi cations, DNA methylation and aberrant expression of microRNAs,

promises better understanding of the biological nature of the disease.

Aim. To issue eff ectiveness and toxicity demetilating medicine (Decitobine) and to fi nd it’s place in standard chemotherapy.

Materials and methods. From 01.2013 to 12.2015 21 patients were enrolled into NII DOG AML 2012 study. The average was 6.5 ± 1.24 years (6 month – 16 years); 12 males and

9 females, standard (SR) – 1 (4.8 %), intermediate (IR) – 6 (28.6 %) and high (HR) – 14 (66.7 %) risk groups. Chemotherapy consisted on 5 curses for HR and IR (AIE, HAM, AI, hAM,

HAE) and 4 curses for SR (AIE, HAM, hAM, HAE). Epigenetic therapy consisted on Valproic acid (VA) days1-78, All Trans Retinoic Acid (ARA) 1–43 days and from the day one to day

14 of the every course chemotherapy and Decitobine in “window” regime before induction (5 patients) and on day 16 after beginning of induction .

Results. Decitobine was used as a demetilating medicine 20 mg/m2 for 5 days in “window” regime before induction (AIE) in fi ve patients. There were no any toxicity and we did not

check decrease of blasts in BM and peripheral blood after Decitobine, one of them developed relapse and one died from severe infection, three pts are alive, and two – underwent by

haploidentical HSCT. Three years DFS was 75 ± 21.7 %, median follow up 30.4 ± 4.8 mo, and EFS – 60 ± 21.9 %, median follow up 24.6 ± 6.4 mo. Thus, Decitobine was moved on the

day 16. Now, 16 pts got this therapy, all of them achieved CR after AIE with VA, ATRA and Decytobine. Two years DFS and EFS were the same – 76.9 % median follow up 24.6 ± 24 mo,

OS – 89.9 ± 10.5 % median follow up 27.1 ± 2.1 mo. None of the patients underwent by allo-HSCT.

Conclusion. The epigenetic therapy increased CR count and survival of children with AML. The place of demetilating agent in AML therapy has to be used in aplasia period after the

fi rst course of induction when patients had got minimum counts of blasts.

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A B S T R A C T N O . : O P - 2 4 8

The impact of conditioning intensity on allogeneic stem cell transplantation outcome

of children and adolescents with acute myeloid leukemia

S.N. Bondarenko1, S.V. Razumova1, O.A. Slesarchuk1, A.L. Alyanskiy1, O.V. Paina1, A.S. Borovkova1, P.V. Kozhokar1,

K.A. Ekushov1, Yu.G. Fedukova1, B.I. Smirnov2, E.V. Semenova1, L.S. Zubarovskaya1, B.V. Afanasyev1

1First Pavlov State Medical University of St. Petersburg, Russia; 2Saint Petersburg Electrotechnical University “LETI”, Russia

Key words: pediatric and adolescent patients, acute leukemia, allogeneic hemopoietic stem cell transplantation, related and unrelated donors, conditioning regimens, donor

lymphocyte infusion

Introduction. Allo-HSCT is the main curative option for the patients with acute myeloid leukemia (AML). The choice of the conditioning regimen intensity may impact on

the allo-HSCT outcome, and still remains the controversial issue.

Aim. The aim was to compare the effi cacy of allo-HSCT in children and adolescents with AML in dependence on conditioning regimen intensity.

Materials and methods. We retrospectively analyzed the data of 118 pediatric and adolescent patients (pts) with AML who underwent allo-HSCT. The median pts age was 12.5

(range 1–21) years. The median follow-up period was 27 (range 2–120) months. At the moment of allo-HSCT 42 (36 %) pts were in the 1st CR, 30 (25 %) in the 2nd CR, 46 (39 %)

had active disease. Thirty one pts (26 %) received allo-HSCT from HLA-matched related, 87 (74 %) – from unrelated donor. The stem cell source was bone marrow in 59 (50 %) and

PBSC in 59 (50 %) allo-HSCT. The myeloablative (MAC, busulfan or treosulfan based) and reduced-intensity (RIC, fl udarabine based) conditioning regimen were used in 66 (56 %)

and 52 (44 %) pts respectively. Acute GVHD prophylaxis consisted of cyclosporine A (n = 79, 67 %) or tacrolimus (n = 39, 33 %).

Thirty pts received donor lymphocyte infusion (DLI) after allo-HSCT. The indications for DLI (n = 35) were disease relapse/progression after allo-HSCT in 26 pts (therapeutic DLI),

minimal residual disease in 4 pts (preventive DLI), and disease relapse prophylaxis in 5 pts (prophylactic DLI). After RIC allo-HSCT more therapeutic (15 vs 11) and less preventive/

prophylaxis (3 vs 6) DLI were used in comparison with MAC allo-HSCT. DLI were combined with additional therapy (chemotherapy (n = 18), hypomethylating agents (n = 9),

interleukin-2 or interferon-a (n = 12)) in 28 pts, or as a monotherapy in 7 pts.

Results. The 5-year OS in MAC and RIC allo-HSCT recipients in the 1st CR was 65 % (95 % CI: 45–85) and 80 % (95 % CI: 59–99) (P = 0.4) respectively. In patients with 2nd CR it was

65 % (95 % CI: 35–95) and 17 % (95 % CI: 1–37) (P = 0.003), in patients with active disease 17 % (95 % CI: 1–43) and 19 % (95 % CI: 1–37) (P = 0.4) respectively. MAC and RIC allo-

HSCT recipients had relapses in 24 % (95 % CI: 10–41) and 29 % (95 % CI: 12–48) (P = 0.7) of cases, respectively. The relapse rate was signifi cantly lower in MAC allo-HSCT recipients

with aGVHD Gr 1–3 compared to RIC allo-HSCT recipients (P = 0.05), although no correlation was observed in patients with cGVHD (P = 0.2). RIC allo-HSCT was associated with

signifi cantly lower toxicity rate, while the infectious complications and GVHD (acute and chronic) rate was comparable for both MAC and RIC allo-HSCT recipients groups.

The CR after DLI was achieved in 12 pts (40 %): in 6 (50 %) pts after RIC and 6 (50 %) pts after MAC allo-HSCT. The median duration of CR after DLI was 5.5 (range 1–38) months.

The relapse occurred in 8 (47 %) out of 17 pts who had CR after DLI.

The mortality rate did not correlate to age or conditioning regimen type. The most common death causes in RIC allo-HSCT recipients were disease progression (36 %), infectious

complications (21 %) and aGVHD (43 %), in MAC – disease progression (37 %), infectious complications (30 %), aGVHD (22 %) and treatment-related toxicity (11 %).

Conclusion. Our results demonstrated the comparable effi cacy of RIC and MAC in children and adolescences with AML in 1 CR and relapse at the moment of allo-HSCT. Effi cacy of DLI

was revealed after both RIC and MAC allo-HSCT.

A B S T R A C T N O . : O P - 2 6 2

High Level of miR-196b at newly diagnosed pediatric

acute myeloid leukemia predicts a poor outcome

Li-hua Xu, Qing Yan, Shaoyan Hu

Children’s Hospital of Soochow University

Key words: miR-196b, AML, pediatric/child, biomarker

Introduction. MicroRNAs (miRNAs) are small non-coding RNAs consisting of ~22 nucleotides, with highly conserved sequence. They widely participate in cellular processes such as

proliferation, diff erentiation, and apoptosis at the post-transcription level. Also, as described having oncogenic or anti-oncogenic properties, miRNAsare implicated in leukemogenesis

and the prognosis, due to some microRNA genes locating in regions of translocations and deletions frequently occurring in leukemia (e.g. miR-15a–miR-16-1 cluster and chronic

lymphocytic leukemia). Moreover, miRNA expression profi les could classify specifi c cancers such as discriminating acute lymphoblastic leukemia (ALL) from AML. Overexpression of

miR-196b has been reported in71 European pediatric AMLwith MLL gene rearrangements, NPM1mutations, as well as FLT3-ITD in cytogenetically normal background.

Aim. To further explore and confi rm the potential function of miR-196b, we investigated the expression of miR-196b and its possible relevant genes SMC1A/MLH1 in 83 Chinese pediatric AML.

Materials and methods. Using quantitative real-time PCR (qRT-PCR), we detected the expression of miR-196b and its correlative genes (SMC1A/MLH1) in initial 83 Chinese pediatric AML.

Results. A signifi cant association was observed between overexpression of miR-196b compared to controls and inferior overall survival of pediatric AML (Log Rank Pission group

after the fi rst induction chemotherapy possessed higher miR-196b expression. Furthermore, a positive relationship was found between the expression of miR-196b and SMC1A/MLH1

(Spearman’s r = 0.37 and 0.44, P = 0.001).

Conclusion. These fi ndings suggest that diff erentially high expression of miR-196b in diagnostic marrow samples of pediatric AML is associated with unfavorable outcome, and miR-

196b potentially can be a novel biomarker for the diagnosis, prognosis and treatment in pediatric AML.

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A B S T R A C T N O . : O P - 2 6 3

The suppression of CCL2 expression decrease the proliferation of HL-60 cells through

downregulation of cyclin D1 via arresting cells at the G1 phase

Jin Yang, Dan Hong, Qi Zhou, Qing Yan, Shaoyan Hu

Children’s Hospital of Soochow University

Key words: chemokine (C-C motif) ligand 2 (CCL2), HL-60 cell line, RNA interference, proliferation, cell cycle, cyclin D1

Introduction. Chemokine (C-C motif) ligand 2 (CCL2) has been demonstrated to be induced and involved in various diseases. In many solid tumors, CCL2 signaling has been shown

to correlate with tumor growth, invasion and metastasis. In hematologic neoplasms, the role of CCL2 has not yet been explicit. There is evidence suggesting that the level of CCL2 has

increased in the bone marrow environment of acute lymphoblastic leukemia (ALL) patients and in the serum of untreated patients with acute myeloid leukemia (AML).

Aim. To explore the mechanism of CCL2 gene on leukemogenesis.

Materials and methods. Lentivirus with CCL2-knockdown was successfully constructed after screening eff ective shRNA sequence and tranfected into HL-60 cells which was

validated on the level of mRNA and protein by qRT-PCR and Western blot, respectively. The cells coming from parental, sh-Vectors and shCCL2 were detected for cell growth viability

by CCK-8 assay, cell cycles and apoptosis by Flow cytometry. We applied exon sequencing technology to identify the gene profi ling between the CCL2 knockdown and the control,

of which, Cyclin D1 was selected for further experiments as it was signifi cantly downregulated. We successfully downregulated Cyclin D1 in HL-60 by means of RNA interference to

detect. Meanwhile, the cell proliferation, cell cycle and apoptosis were detected by CCK-8 assay, Flow cytometry, respectively.

Results. After suppressing CCL2 in HL-60 transfected cells, the cell proliferation rate decreased at the most of 44.44 % compared to control cells. Cell cycle analysis demonstrated

that 12 % more cells arrested at G1 phase. Gene expression profi ling data revealed there are total 159 diff erentially expressed genes related to cell cycle, NOD-like receptor signaling

pathway, TNF signaling pathway and NF-kappa B signaling pathway including Cyclin D1 which was downregulated by 60 %, meanwhile, Cyclin D1 was decreased on the level

of mRNA and protein. Moreover, the shRNA-mediated inhibition of Cyclin D1 suppressed cell growth and blocked more cells at G1 phase, which was consistent to that of CCL2 silencing.

Conclusion. Downregulation of CCL2 inhibits the cell proliferation which was mediated by suppressing cyclin D1 via blocking more cells at G1 phase.

A B S T R A C T N O . : O P - 2 6 5

High expression of S100A8 gene is associated with drug resistance to etoposide via

infl uencing apoptic genes expression and poor prognosis in acute myeloid leukemia

Xiaoyan Yang, Mingying Zhang, Qi Zhou, Shaoyan Hu

Children’s Hospital of Soochow University

Key words: S100A8, HL-60 cell, drug resistance, acute myeloid leukemia (AML), prognostic factor, anti-apoptosis

Introduction. S100A8 have been increasingly recognized as biomarkers in multiple solid tumors and played pivotal roles in hematological malignancies. S100A8 is potentially

an indicator for poor survival in acute myeloid leukemia (AML) in retrospective studies. However, the mechanisms of S100A8 are diverse in cancers.

Aim. In this study we investigated the correlation of S100A8 at the transcription level with clinical parameters in AML patients and explored its chemoresistance mechanism in vitro.

Materials and methods. A total of 189 AML patient specimens from diff erent treatment stages of AML underwent induction chemotherapy between 2010 to 2014 was included.

Among them, 91 were de novo AML patients (excluded secondary AML, therapy related AML or AML evolved from antecedent haematological disorders), 64 patients in complete

remission (CR) and 34 patients in relapse, in addition to 20 controls without leukemia. S100A8 transcription level was detected by real-time RT-PCR and normalized to β-actin

transcription level. Lentivirus vector with S100A8 overexpressed was constructed and transfected into HL-60 cell line. Biological characters and resistance to chemotherapy drugs

were compared between transfected cells.

Results. The transcription levels of S100A8 was signifi cantly associated with cytomorphological classifi cation, the results showed that S100A8 transcription levels were higher in

FAB M4 and M5 than in the M0 and M1 subtypes (P = 0.042). Patients with high and low S100A8 transcription levels had median OS times of 15 (IC: 12.8, 20) and 23 (IC: 18.5, 23.5)

months, respectively (P = 0.024). The estimated 3-year OS rates of high expression S100A8 group was 32 % (IC: 16 %, 52 %) versus 59 % (IC: 41 %, 76 %) for the low expression

group (P = 0.042). Based on multivariate model for OS, high S100A8 transcription was a signifi cant prognostic factor (P = 0.003) after adjustment for age (P = 0.025), bone marrow

blast percentage (P = 0.016) and cytogenetic classifi cation (P = 0.039) at diagnosis. Overexpression of S100A8 caused etoposide resistance in a dose and time dependent manner.

Using real-time apoptosis PCR arrays, we analyzed and clustered the expression of 370 genes associated with apoptosis pathway in control and S100A8 overexpression cells treated

by etoposide. Thirty six genes were signifi cantly downregulated and 12 genes were signifi cantly upregulated in S100A8 overexpression group compared with control group, of which

the caspase-3, Bcl-2 and Bax was verifi ed by western blot analysis.

Conclusion. Critical S100A8 provided useful clinical information in predicting the outcome of AML. One of the mechanisms was that S100A8 involved in chemoresistance via anti-

apoptosis way.

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A B S T R A C T N O . : O P - 2 7 2

Abandonment and outcome of childhood acute myeloid leukaemia

in a tertiary level hospital

Momena Begum, Chowdhury Yakub Jamal, Afi qul Islam, ATM Atikur Rahman, Mehnaz Akter,

Chowdhury Shamsul Hoque Kibria, Rasel Siddique, Lutfor Rahman Molla, Zannat Ara

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Key words: abandonment, outcome, childhood acute myeloid leukemia

Introduction. Acute leukemias are the most common child hood malignancy, of which acute myeloid leukemia (AML) are 15 % to 20 %. Abandonment is one of the most important

causes of treatment failure in AML in developing countries. Lost to follow-up is also a big problem in low income countries . Many patients stop therapy soon after diagnosis due

to cost, distance and ignorance.

Aim. To determine the abandonment, outcome and treatment related mortality (TRM) and morbidity among children with AML.

Materials and methods. This prospective observational study was conducted in the Department of Pediatric hematology and Oncology, BSMMU, Dhaka for duration of one year.

Fifty (50) patients of AML visited to out patient department (OPD) of Pediatric hematology and Oncology. Among them 11 (22 %) patients refuse treatment from outdoor. Thirtynine

39 (78 %) patients of AML were selected as per inclusion and exclusion criteria. After proper evaluation and clinical examination of these patient, CBC and Bone marrow examination

was done for confi rmation of diagnosis.

Results. A total of 39 patients were recruited in this study. 17 (43.6 %) patients were male and 22 (56.4 %) were female. Mean ± SD of age was 7.80 ± 4.42 and range was 6 months

to 15 years. Out of 39 patients, 18 (46.1 %) patients were abandoned, 15 (38.4 %) expire, relapse 2 (5.2 %) & alive 4 (10.3 %).

Conclusion. High abandonment (46.1 %) and treatment related toxic death (38.4 %) has compromised the outcome of Acute myeloid leukemia. However AML can be treated with

better outcome if improved the supportive care, reduce toxic death, refusal or abandonment.

A B S T R A C T N O . : P P - 2 8 9

Crosstalk between THP-1 and MSC changes adipocyte and osteoblast diff erentiation

Moon Kyu Kim1, Kyoung-Mi Lee2, Kiwon Park2, Seung Min Hahn1, Jung Woo Han1, Chuhl Joo Lyu1

1Yonsei University College of Medicine; 2Yonsei University BK21 plus

Key words: human bone marrow derived, mesenchymal stromal cells, adipogenesis, osteogenesis, acute myeloid leukemia

Introduction. Leukemic cells can acquire chemo-resistance during therapy and human Bone marrow derived mesenchymal stromal cells (hBMSCs) are one of the key players

in this process. The crosstalk between leukemic cells and BMSCs results in changes of characteristics in both cells.

Aim. We investigated the eff ects of THP-1 on diff erentiation potency of MSCs when co-cultured.

Materials and methods. Bone marrow aspirates were obtained from the posterior iliac crest of 10 healthy adult donors ranging from 20–69 years of age under the approval

of the Institutional Review Board (IRB). The BMSCs from healthy donor’s BM was co-cultured with THP-1 in DMEM. To identify the eff ects of THP-1 cells dosage on BMSC diff erentiation

potency, the 3X10e4, 6X10e4, and 1X10e5 of THP-1 cells were added in 1X10e5 of BMSCs cells. To confi rm the osteogenic and adipogenic diff erentiation ability of hBMSC by THP-1, we

co-cultured BMSCs and THP-1 cells in osteogenic and adipogenic medium. Osteogenic medium consisted of complete growth medium plus osteogenic growth supplements comprised

of 50 μM ascorbic acid, 0.1 μM dexamethasone, and 10 mM β-glycerophosphate and adipogenic medium consisted of DMEM-low glucose supplemented with 1 % antibiotic–

antimycotic solution plus MDI (0.5 mM isobutylmethylxanthine, 1 mM dexamethasone, and 200 mM indomethacin, and 5 mM insulin. After 14 days, osteogenic and adipogenic

diff erentiation were determined by Alizarin red S and Oil-Red-O staining respectively.

Results. At diff erentiation condition, the THP-1 cells were attached to hBMSCs during osteogenesis, but less in adipogenesis. The co-culture of THP-1 cells and BMSCs had enhanced

diff erentiation ability when compared to diff erentiation of BMSCs alone. The osteogenic and adipogenic diff erentiation potency of BMSCs were increased when the number of THP-1

cells were increased in dose dependent manner.

Conclusion. Our results suggest that the THP-1 cells eff ects on adipogenic and osteogenic diff erentiation potency of hBMSCs, and these changed potency might augment chemo-

resistant characteristics of THP-1 cells. To identify our expectation, the further study is required such as the eff ect of leukemic cells from diff erent lineage and hBMSCs from various

origin.

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A B S T R A C T N O . : P P - 3 0 1

Prognostic signifi cance of molecular genetic changes

in childhood acute myeloid leukemia

Yu. Barouskaya, M. Stsiohantsava, A.M. Kustanovich, O.I. Bydanov, O.V. Aleinikova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: acute myeloid leukemia, molecular genetic changes, prognosis

Introduction. The most important prognostic factors in acute myeloid leukemia (AML) are molecular genetic changes in blast cells and response to treatment.

Aim. An analysis of prognostic signifi cance of molecular genetic changes in AML in children, that received treatment in the Republic of Belarus.

Materials and methods. 151 patients aged 0.02 to 25.8 years are included in the study (median age – 10.75 years) with de novo AML, that received programmed treatment

in Republican research and practice centre of pediatric oncology, hematology and immunology (Minsk) from May 1999 to December 2013 according to original protocols AML-ММ-2000

(n = 81) and AML-ММ-2006 (n = 70). Patients treatment results are defi ned on 01.02.2016. Follow-up median was 1.75 years. A cytogenetic analysis was performed in a common

method of short-term bone marrow cell cultivation with further diff erentiated G-staining of metaphase chromosome, as well as by method of fl uorescence in situ hybridization (FISH).

A method of reverse transcription polymerase chain reaction was used to detect chimeric oncogenes. Prescrining by method of SSCP (Single Strand Conformational Polymorphism)

was performed to detect mutations in CEBPA and NPM1 genes. The results were confi rmed by method of direct Sanger sequencing.

Results. A cumulative incidence of relapse (CIR) in patients with CBF-leukemia (inv(16) and t(8;21)) was 8.9 ± 4.9 % in comparison with the ratio 35.8 ± 4.5 % in the remaining/

rest cohort of patients, P = 0.0030. In 11q23-anomalies group t(1;11)(q21;q23) was detected only in 2 patients. Both of the children are in the fi rst long-term remission without

performing of hematopoietic stem cell transplantation. CIR in the group with t(9;11)(p22;q23) appeared to be lower than in patients with t(10;11)(p12;q23) (18.8 ± 10.2 % и 58.3 ± 19.3 %,

respectively, P = 0.0384) and in patients with t(6;11)(q27;q23) (33.0 ± 25.6 %, P = 0.7026). In the investigated/studied cohort t(2;11)(p21;q23) was reported only in 1 patient

with congenital AML that died in induction. In the group of patients with normal karyotype (NK) where no additional genetic event was reported by accessible methods, CIR was

44.4 ± 12.4 %. However CIR in the group of patients with NK and CEBPA and NPM1 gene mutations appeared to be signifi cantly lower compared to a similar ratio in patients with NK

and FLT3 mutation (16.7 ± 16.7 % and 100 %, respectively, P = 0.0254).

Conclusion. The presence of inv(16) и t(8;21) in AML was associated with a favorable outcome. CIR ratios in patients with t(1;11) are comparable to CBF group, that also assigns

this anomaly to prognostically favorable. Relapse development risk in patients with t(10;11) is higher than in the remaining cohort with 11q23. Prognostic infl uence on a disease

outcome in patients with NK is made by the absence or presence of additional genetic events. The presence of CEBPA or NPM1 mutations determines a favorable prognosis, whereas

FLT3 mutation – adverse prognostic factor.

For improvement of stratifi cation of this cohort of patients with AML a detection of additional genetic prognostic factors is required.

A B S T R A C T N O . : O - 3 1 5

Acute myeloid leukemia as a second disease. Belorussian experience

Yu. Barouskaya, O.V. Aleinikova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: acute myeloid leukemia, second disease, children

Introduction. A defi nition «acute myeloid leukemia (AML) as a second disease» is not clear. According to World Health Organization AML, appeared in patients, that received cytotoxic

chemotherapy and/or radiation therapy for malignant or non-malignant disease is qualifi ed as AML, associated with previous treatment (tAML). AML development in patients with

non-malignant hematopoietic diseases (ex., aplastic anemia (АА)) is rarely reported. Secondary AML (sAML) is defi ned as AML associated with previous myelodysplastic syndrome

(MDS) or myeloproliferative disease. Generally development of AML as a second disease is signifi cant adverse prognostic factor.

Aim. To evaluate molecular genetic specifi cs and treatment results of patients with AML, developed after myeloid neoplasm (MNO) and AA therapy.

Materials and methods. Fourteen patients received treatment in our clinic for fi rst and second disease from 1999 to 2015. Girls were predominant (boys/girls – 0.27). Median

age at AML was 13.8 years (range – 6.3–20.8). In 8 (57 %) patients AML developed after MNO (3 cases of osteosarcoma, 2 – acute lymphoblastic leukemia, 1 – rhabdomyoblastoma,

1 – non-Hodgkin lymphoma, 1 – Hodgkin lymphoma); in 5 (36 %) – after acquired AA treatment; in 1 case – transformation from Fanconi anaemia. Median time from the moment

of the fi rst disease to AML development was 2.12 years (range – 0.8–8.8). AML treatment was performed according to protocols for de novo AML. Medan follow-up was 1.34 (0.002–10.9) years.

Results. In all patients (5/5) from the group with acquired AA anomalies of chromosome 7 were detected. In the development of AML a complex karyotype was found in patient with

Fanconi anemia. In 5 of 8 patients from the group of MNO 11q23 anomalies were shown.

The probability of 15-years overall and event-free survival for the whole cohort of patients was 47 ± 14 % and 43 ± 13 %, respectively. In groups with fi rst MNO and AA there were

no signifi cant diff erences. Induction failures were admitted in 4 (28.6 %) patients, death in remission – in 2 (14.3 %), relapse – in 1 (7 %) patient. In 1 patient fi rst tumor progression

(rhabdiosacioma) was reported after AML remission. Hematopoietic stem cell transplantation was performed in 7 patients (3 patients from the group with acquired AA, 4 – from

the group of MNA). EFS after HSCT was 71 ± 17 %. 3 (100 %) patients from the group with acquired AA are alive after HSCT, 2 (50 %) patients from the group of MNA died from the

posttransplantational complications.

Conclusion. With tAML development in the structure of fi rst disease MNO are dominant (osteosarcoma is in the lead). Eff ective tAML treatment method (especially after AA)

is HSCT. In fi rst AA diagnostics it is necessary to undertake a cytogenetic study for monosomy 7, thereby performing a diff erentiated diagnostics with MDS and selecting potential

candidates for HSCT.

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A B S T R A C T N O . : P P - 0 9 9

The effi cacy of cyclosporin in the treatment of infant case with subcutaneous

panniculitis-like T-cell lymphoma (SPTCL)

Michihiro Yano, Miwa Hebiguchi, Koya Kodama, Tsutomu Takahashi

Akita University Hospital

Key words: subcutaneous panniculitis-like T-cell lymphoma, cyclosporin, immunosuppressive therapy, combination chemotherapy

Introduction. SPTCL is an extremely rare type of cutaneous lymphoma in infants. We herein present a case of pediatric SPTCL that showed a good response to immunosuppressive

therapy with cyclosporine (CyA).

Aim. This case is special for two reasons: fi rst, it is the youngest reported case of SPTCL in the literature; and second, we obtained a desirable treatment response with CyA in spite

of the patient’s poor response to combination chemotherapy.

Materials and methods. Case. A 1-month-old male presented with multiple erythematous subcutaneous nodules on his earlobe, cheek, hips, and upper and lower extremities.

He was diagnosed with SPTCL based on the characteristic pathological fi ndings in a skin biopsy specimen. Because he had no clinical fi ndings except broad skin involvements,

we initially managed him with prednisolone monotherapy. Although we observed a rapid improvement of his skin disease, a few nodules persisted and gradually spread

as the prednisolone dose was tapered. We then administered combination chemotherapy consisting of vincristine, cyclophosphamide, doxorubicin and prednisolone (CHOP).

During the administration of prednisolone his skin nodules improved, but it worsened as soon as the prednisolone treatment was terminated. We concluded that the combination

chemotherapy was not eff ective for treating his disease.

Results. Based on the fact that prednisolone was partially eff ective in treating the patient’s disease, we considered that immunosuppressive agents such as cyclosporine

and tacrolimus might aff ect his skin disease. He was therefore treated with CyA (6 mg/kg/day, orally). All of his subcutaneous nodules disappeared and did not reemerge during drug

tapering.

Conclusion. The standard treatment for SPTCL is multi-agent combination chemotherapy. It is unclear why combination chemotherapy was not eff ective in this case. It is possible

that the dormancy of the SPTCL cells may be related. Therefore, immunosuppressive treatments with CyA for SPTCL may be eff ective when combination chemotherapy is ineff ective.

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A B S T R A C T N O . : P - 1 0 3

Modern aspects of diff erential diagnosis and treatment of large B-cell lymphomas

with mediastinal involvement in children and adolescents

A.S. Levashov, A.M. Kovrigina, A.M. Stroganova, T.T. Valiev, A.V. Popa

N.N. Blokhin Russian Cancer Research Center, Moscow, Russia

Key words: large B-cell lymphomas, mediastinal involvement, children, diff erential diagnosis, molecular-biological markers

Introduction. At the present time results of some pediatric protocols (FAB/LMB96 with and without rituximab, B-NHL-BFM90/95, B-NHL-2004m) were published but prognostic

and diagnostic signifi cance of markers, such as: C-MYC, STAT3, pSTAT3tyr705, TRAF1, TNFAIP2 expression, C-MYC gene rearrangement and amplifi cation in childhood large B-cell

lymphomas with mediastinal involvement (diff use large B-cell lymphoma – DLBCL, primary mediastinal (thymic) large B-cell lymphoma – PMLBCL) is unknown.

Aim. The aim of this study was to determine distinctive molecular-biological features of childhood large B-cell lymphomas with mediastinal involvement, to estimate the signifi cance

of these markers for overall survival (OS), event-free survival (EFS) and progression-free survival (PFS).

Materials and methods. From 1994 to 2015 twenty two pediatric patients with large B-cell lymphomas with mediastinal involvement were included in trials IDM-NHL-BFM90,

B-NHL-BFM 95 ± rituximab (10 with DLBCL, 12 with PMLBCL). Male/female ratio was 1/2. Median age – 12.2 ± 0.6 years (range from 5 till 18). Minimal age of patients with

DLBCL was 5 years, for patients with PMLBCL – 10 years. Stage III-IV, R3-R4 risk groups were revealed in all patients (100 %). GCB/non-GCB DLBCL subtypes were assessed by Hans

and Visco-Young immunohistochemical algorithms. Cutoff values of 40 % for MYC, 70 % for BCL2, 20 % for STAT3, 50 % for pSTAT3tyr705, TRAF1, TNFAIP2 were established. MYC gene

rearrangement and amplifi cation were assessed by FISH using locus-specifi c MYC (8q24) tricolor break apart probe and MYC (8q24) SE8 control probe.

Results. Molecular portrait of childhood primary mediastinal (thymic) large B-cell lymphoma was characterized by: CD20 expression in all patients, high frequency of CD23, CD30,

C-MYC, PAX5, TRAF1, TNFAIP2 expression, absence of CD10, p-STAT3tyr705 expression, low frequency of IgM expression, absence of C-MYC gene rearrangement and/or amplifi cation

despite the level of C-MYC expression more 70 %, high activity of JAK2-JMJD2C epigenetic regulation of p-STAT3tyr705-independent C-MYC expression. While molecular features

of childhood diff use large B-cell lymphoma with mediastinal involvement included: CD20 expression in all patients, non-GCB variant, high frequency of C-MYC expression, C-MYC gene

rearrangement, absence of CD23, TRAF1, TNFAIP2 expression, low frequency of CD30 expression.

Using protocol B-NHL-BFM-95 plus rituximab we have achieved high level of progression-free survival in patients with large B-cell lymphomas with mediastinal involvement (100 %,

a median follow-up was 50 ± 16.7 months; only for patients who completed whole program therapy). The infl uence of investigated markers (CD23, CD30, C-MYC, STAT3, p-STAT3tyr705)

on OS, EFS and PFS was not observed.

Conclusion. Number of patients in this study is not suffi cient for estimation of prognostic signifi cance of these markers but high-intensive B-NHL-BFM-95 + rituximab chemotherapy

showed good therapeutic eff ect in our patients. This study will be continued.

A B S T R A C T N O . : P P - 1 0 6

Cytogenetic adverse prognostic factors in childhood Burkitt lymphoma

A.S. Fedorova, E.V. Volochnik, R.I. Yutskevich, O.V. Aleinikova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: Burkitt lymphoma, cytogenetic, prognosis

Introduction. Inspite of good treatment results of children with Burkitt lymphoma (BL) and low recurrence rate, prognosis for patients (pts) with disease failure is dismal. Therefore

search for relapse predictors remains actual. There are few reports concerning adverse prognostic value of such chromosomal abnormalities as del13q32, gain 7q, dup(1)(q12q32)

in limited cohorts of pts.

Aim. We aimed to analyze BL cytogenetic fi ndings and their prognostic signifi cance.

Materials and methods. A total of 56 BL pts from 2 to 23 years old (median age, 8) diagnosed in our hospital in 1998–2015 were studied. M/F ratio was 5.2/1. Three pts had stage

I tumor, 6 – II, 14 – III, 9 – IV, 24 – leukemia. Pts were treated according to consecutive BFM-based regimen (modifi ed NHL-BFM-90/95, B-NHL-M-2004/2010 (Russia)). Probability

of 5-y EFS was 0.75 for all studied pts. Chromosomal analysis was performed using conventional cytogenetic analysis (G-banding) in 53 newly diagnosed and in 3 relapsed pts with BL.

Results. Detection of dup(13)(q14q32) was associated with primary abdominal site (in 5 of 6 cases) and high relapse rate of 66.7 % (pf 10 and 2 years (stage IIR in 1pt; stage

IV (BM + CNS) in 1 pt, who received high dose chemotherapy and autologous heamotopoietic stem cell transplantation after the 5th course of conventional chemotherapy).

Monosomy of chromosome 17 was detected in 4 pts, all of them died of tumor progression (P = 0.00001).

Conclusion. Karyotypes of BL cells showed a high degree of diversity and complexity. Detection of dup(13)(q14q32) or monosomy 17 are associated with unfavorable prognosis.

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A B S T R A C T N O . : P - 1 4 5

Hodgkin lymphoma in young adults

S.A. Kulyova, A.P. Karitsky, S.V. Ivanova

N.N. Petrov Research Institute of Oncology, Saint Petersburg, Russia

Key words: young adults, Hodgkin’s lymphoma, survival rate

Introduction. Young adults represent a unique cohort of the population with high level of malignant tumors incidence. Hodgkin’s lymphoma (HL) makes about 12 % of all

tumors at 19–29-year-old patients. Young adults isn’t rather investigated since treatment it is carried out in various options according to diff erent programs (pediatric and adult),

and the remote consequences of therapy in such patients aren’t studied.

Aim. The purpose of our investigation was to assess of therapy for Hodgkin’s lymphoma in young adults.

Materials and methods. From January 1995, to July 2011, 87 patients aged from 19 to 29 years old (median 24 ± 4 years old) with biopsy-proven HL were treated

at the N.N. Petrov Research Institute of Oncology. There were 34 (39.1 %) men and 53 (60.9 %) women (the male-to-female ratio was 2.1 : 1). B symptoms occurred in 47 patients

(54 %). There were 47 patients (54 %) with clinical stage I–II and 40 (46 %) – with stage IV. Extranodal involves is revealed in 37 patients (42.5 %). The distribution of the HL according

to the histological classifi cation was as follows: 76 patients (87.4 %) had nodular sclerosis, 5 (5.7 %) – mixed cellularity, 2 (2.3 %) – lymphoid predominant type and 4 patients

(4.6 %) were unclassifi ed due to unsuffi cient quality of the specimens obtained. All patients were given 2–11 cycles of primary chemotherapy (median 4 cycles) with ABVD

(48 patients, or 55.2 %), BEACOPP (36, or 41.4 %), MOPP (2, or 2.3 %) and COPP (one patient). Chemotherapy regimen was followed by extend- or involved- or subtotal radiation with

doses from 20 to 51 Gy in 57 patients (65.5 %).

Results. All patients were assessed for the treatment results. With a median of 31 months of follow-up (range, from 3 to 156 months) the freedom from treatment failure of 3 years

after diagnosis was 47.4 % (SE – standard error – 7.8 %), event-free survival was 70.5 % (SE 7.2 %), and overall survival was 82.7 % (SE 5.2 %). Of the 72 patients who received

the therapy and achieved a complete remission, 19 (21.8 %) relapsed at a median time of 31 months from initial diagnosis (range, 7 to 80 months).

Conclusion. Low survival in young adults with HL confi rm need of change of combined modality therapy role with transition on using principle of risk-adapted treatment that will

allow to achieve good results, and to decrease side eff ects of follow-up.

A B S T R A C T N O . : P - 1 4 6

The infl uence of “bulky” disease on survival in patients with Hodgkin lymphoma

S.A. Kulyova, A.P. Karitsky, S.V. Ivanova

N.N. Petrov Research Institute of Oncology, Saint Petersburg, Russia

Key words: young adults, Hodgkin lymphoma, “bulky” disease

Introduction. About 70 % of patients with the Hodgkin lymphoma (HL) may be cured. The analysis of various predictive factors is applied to identifi cation of the remained patients

who need intensive therapy. “Bulky” disease is the most important parameter which directly has impact on survival of patients with malignant tumors.

Aim. The aim of the study was the assessment of tumor burden’s components (a mediastinum/thoracic ratio – MTR, peripheral bulky disease, and number of involved lymph nodes)

in young adults with Hodgkin lymphoma and the analysis of their predictive value.

Materials and methods. Data on 87 patients aged from 19 till 29 years (median 24 ± 4 years) are submitted. The male-to-female ratio was 2.1 : 1. B symptoms occurred

in 47 patients (54 %). There were 47 patients (54 %) with clinical stage I-II and 40 (46 %) – with stage IV. Extranodal involves is revealed in 37 patients (42.5 %). The distribution of

the HL according to the histological classifi cation was as follows: 76 patients (87.4 %) had nodular sclerosis, 5 (5.7 %) – mixed cellularity, 2 (2.3 %) – lymphoid predominant type and

4 patients (4.6 %) were unclassifi ed due to unsuffi cient quality of the specimens obtained.

Results. The optimum cut-off level according ROC method for MTR has been established at 0.33, for peripheral bulky disease has been established at 46 mm, and for number

of involved lymph nodes has been established at 5. Use of the correlation and regression analysis allocated the best linear regression model for MTR with the maximum determination

coeffi cient and the minimum residual dispersion (P = -0.928971 + 4.008252x). There were statistically signifi cant the equation by Fischer’s criterion and determination coeffi cient

(Ffact(46.74) > Ftheor(4.17)). Linear and nonlinear regression models for “a peripheral lymphadenopathy” and “number of involved zones” didn’t show signifi cant infl uence on the

outcome (P = 0.9237 and P = 0.3385, respectively). MTR signifi cant infl uenced the late results. The disease-free survival in patient’s group with MTR less than 0.33 was 100 %,

in group with MTR more than 0.34 was 41 % (log-rank test 0.04649).

Conclusion. The study proved that mediastinum/thoracic ratio is the signifi cant prognostic factor. Such factors as “a peripheral lymphadenopathy” and “number of involved zones”

had smaller predictive value. These components are indicators of “bulky” disease and demand further studying.

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A B S T R A C T N O . : P - 1 4 7

Pair linear dependence of prognostic factors with a tumor volume in young adults

with Hodgkin’s lymphoma

S.A. Kulyova, A.P. Karitsky, S.V. Ivanova

N.N. Petrov Research Institute of Oncology, Saint Petersburg, Russia

Key words: Hodgkin’s lymphoma, tumor volume, young adult

Introduction. Hodgkin’s lymphoma in young adult is an aggressive disease with poorer prognosis than in childhood.

Aim. The aim of study was creation of the pair linear dependence equations of prognostic factors with factor «the tumor volume».

Materials and methods. 87 patients aged from 19 to 29 years old (median 24 ± 4 years old) with Hodgkin’s lymphoma were treated at the N.N. Petrov Research Institute of Oncology

in 1995–2011. The male-to-female ratio was 2.1 : 1: there were 34 (39.1 %) men and 53 (60.9 %) women. Forty-seven (54 %) had B symptoms. There were 47 patients (54 %) with

clinical stage I–II and 40 (46 %) – with stage IV. Thirty-seven patients (42.5 %) had E-stage. The distribution of the HL according to the histological classifi cation was as follows:

76 patients (87.4 %) had nodular sclerosis, 5 (5.7 %) – mixed cellularity, 2 (2.3 %) – lymphoid predominant type and 4 patients (4.6 %) were unclassifi ed. All patients were given 2–11

cycles of primary chemotherapy (median 4 cycles) with ABVD (48 patients, or 55.2 %), BEACOPP (36, or 41.4 %), MOPP (2, or 2.3 %) and COPP (one patient). Chemotherapy regimen

was followed by extend- or involved- or subtotal radiation with doses from 20 to 51 Gy in 57 patients (65.5 %).

Results. The pair correlation method was carried out for an assessment of narrowness, a vector and analytical expression of parameters communication with a tumor

volume. Haemoglobin (y[haemoglobin] = - 1.407407 + 0.259259x[volume], r = 0.018730), fi brinogen (y[fi brinogen] = 1.250000 + 0.350000x[volume], r = 0.028944),

LDG (y[LDG] = 0.85185 + 0.148148x[volume], r = 0.020156) and no eff ective therapy (y[“-”eff ect] = 0.370370 + 0.387205x[volume], r = 0.000332) and B-symptoms (y[B-symptoms] =

1.413043 + 0.232118x[volume], r = 0.046422) had signifi cant communication with tumor volume.

Conclusion. Allocation of pair correlation and creation of correlation model allows to consider these factors as possessing a multicollinearity, they can be intercorrelated and excluded

from the fi nal multifactor analysis.

A B S T R A C T N O . : P - 1 5 7

Positron emission tomography in diagnosis of malignant lymphomas in children

I.I. Badrtdinova1, R.R. Bayramgulov1, O.N. Lipatov2, D.A. Sinilo1 1Republican Children’s Clinical Hospital, Ufa, Republic of Bashkortostan;

2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: malignant lymphomas, children, positron emission tomography/computed tomography with 18F-fl uorodeoxyglucose, diagnosis

Introduction. Malignant lymphomas in children are a heterogenous group of diseases in the context of which treatment and prognosis are determined by adequate staging and

follow-up/observation. A standard imaging procedure is computed tomography (CT). Positron emission CT with 18F–fl uorodeoxyglucose (PET/CT with 118F-FDG) can play a promising

role in the diagnosis of malignant lymphomas in children.

Aim. To investigate the role of PET/CT with 18F-FDG for staging, treatment planning and response assessment in children with malignant lymphomas.

Materials and methods. During the period 2014–2015 in the Republic of Bashkortostan, 34 PET/CT with 18F-FDG imaging exams were performed in 20 children (girls – 6, boys – 14; all

children aged 0–18 years old) with histologic diagnosis “malignant lymphoma” (Hodgkin lymphoma (n = 12), Burkitt lymphoma (n = 3), anaplastic large cell lymphoma (n = 2),

T-lymphoblastic lymphoma (n = 3), B-lymphoblastic lymphoma (n = 1)). Investigations were performed both for disease staging, intermediate assessment («response-controlled»

treatment) and for the control of treatment. 18F-FDG PET/CT fi ndings were compared with the results of CT with intravenous contrast (IV contrast). Tumour process extent was

evaluated based on the analysis of 18F-FDG PET/CT fi ndings, CT scans with IV contrast only as well as fi ndings of 18F-FDG PET/CT and CT with IV contrast performed simultaneously.

Inclusion criteria for the investigation were: the availability of protocols and medical images (CT with IV contrast, PET/CT with 18F-FDG). The investigation was performed only

for FDG-sensitive lymphomas. The period of the investigation was limited to the period of follow-up after the performed investigations (median follow-up duration – 12 months).

Results. PET/CT with 18F-FDG is a more eff ective method for detection of lymphatic tissue involvement (sensitivity – 93 %, specifi city – 100 %) in comparison with CT with IV contrast

(sensitivity – 88 %, specifi city – 86 %). In case of extranodal involvement, this method also has the advantage (sensitivity – 88 %, specifi city – 100 %) over CT with IV contrast

(sensitivity – 50 %, specifi city – 90 %). The analysis of patients who underwent simultaneously performed PET/CT with 18F-FDG and CT with IV contrast showed the best result, proper

treatment tactics was accepted in 100 % of cases, and this rate was higher than in case of CT with IV contrast (35 %).

Conclusion. PET/CT with 18F-FDG should be applied for staging of FDG-avid lymphomas, detection of residual foci in lymph nodes and extranodal involvement during the treatment

as well as when disease recurrence is suspected. CT with IV contrast should be applied in the staging of FDG-negative lymphomas. Simultaneous performance of 18F-FDG PET/CT

and CT imaging with IV contrast is considered reasonable for optimal anatomical assessment of involved areas, adequate staging and control of treatment process.

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A B S T R A C T N O . : P - 1 9 9

Intensive therapy children with advansed stage Hodgkin’s lymphoma

E.S. Belyaeva

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: Hodkin’s lymphoma, BEACOPP, advansed stage

Introduction. The treatment results of children with local stage of Hodgkin’s lymphoma (LH) are not bed, but the treatment results patients with advanced stage of LH aren’t satisfi ed.

Aim. We decided to study effi cacy and toxicity of chemo (escBEACOPP).

Materials and methods. From 2003 to 2015 103 pts (female 53, male 50) with advanced stage LH were enrolled in this study. The median age was 12.5 y. (3–18). Primary

staging was: stage II – 34 pts (33 %) with bulk disease (mediastinal mass greater than 1/3 of the mediastinal thoracic diameter and/or nodal aggregate greater than 10 cm), stage

III – 23 pts (22.3 %), stage IV – 46 pts (44.7 %). Therapy induction consisted on 4 courses of escBEACOPP (Cyclophosphamide – 1200 mg/m2/d, days 1, Etoposide – 200 mg/m2/d, days

1–3, Doxorubicin 35 mg/m2/d, on day 1, Bleomycin 10 U/m2/d, days 8, Vincristine 1,5 mg/m2/d, days 8, Procarbasine 100 mg/m2/d, days 1–7, Prednizone 20 mg/m2/d, days 1–14).

Consolidation was diff erent for females and males and also depended on response to 4 courses of indaction. Females with a good response to therapy (CR, PR1 – 70 % reduction

in disease) received 4 courses COPP/ABV (Cyclophosphamide – 600 mg/m2/d, days 1, Doxorubicin 35 mg/m2/d, on day 8, Bleomycin 10 U/m2/d, days 8, Vincristine 1,5 mg/m2/d, days

1, Vinblastine 6 mg/m2/d, days 8, Procarbasine 100 mg/m2/d, days 1–7, Prednizone 20 mg/m2/d, days 1–14) without RT. Male with CR or PR1 received 2 courses ABVВ (Doxorubicin

25 mg/m2/d, on days 1, 15, Vinblastine 6 mg/m2/d, days 1, 15, Bleomycin 10 U/m2/d, days 1, 15, Dacarbazine 375 mg/m2/d, days 1, 15) with involved fi eld RT 20 Gy.

Results. Complete response after 4 courses got 32 pts (31.1 %), PR1 – 55 pts (53.4 %), PR2 – 15 pts (14.6 %), SD – 1 pts. Thirty-nine females haven’t got RT (3 – developed relapse

with a still but in second CR).

Median follow-up is 145.0 ± 2.4 mo, RFS = 91.5 ± 2.9 %; EFS = 89.6 ± 3.1 %; OS = 97.0 ± 1.7 %.

In spite of severe hematological toxicities – grade IV 45.6 % just two patients had fatal infection and one grade III mucositis. There were no any non- hematological toxicity.

Conclusion. We suggest that escBEACOPP regimen is tolerable and shows good treatment results in patients with advanced stages HL.

A B S T R A C T N O . : P - 2 1 9

Burkitt’s lymphoma in children: Is a second CVP eff ective in critically ill children?

Emad A.H. Moussa1, Asmaa M. Hamoda2, Samah F. Semary3, Marwa Romeih4, Randa Amin5, Omneya Hassanin6

1Dept Clinical Oncology-Menofi ya University, Consultant PHO at CCHE; 2Dept Pediatric Hematology/Oncology-NCI – Cairo University, Consultant PHO at CCHE; 3Pediatric Oncology-Faculty of Medicine-Beni Suef University, and Consultant PHO at CCHE;

4Radiodiagnosis –NCI – Cairo University, Consultant Radiodiagnosis at CCHE;5Pathology-Faculty of Medicine-Assiut University, and Consultant pathology at CCHE; 6CRA at CCHE

Key words: Burkitt’s lymphoma, pediatrics, induction chemotherapy

Introduction. Non-Hodgkin lymphoma is the fourth most common malignancy in children, has an even higher incidence in adolescents. A progress has been achieved, with survival

rates exceeding 80 %. Patients with advanced-stage disease are at signifi cant risk of roughly 3 % to die from treatment related complications, especially during the fi rst phase

of therapy. Protecting those patients from the acute and/or chronic toxicity of antineoplastic therapy has become a major concern of oncology centers around the world.

Aim is to analyze the outcome of those critically ill patients who could not continue to have Induction phase and were given a second CVP, and to measure the delay period before

which those patients started their fi rst COPADM course.

Materials and methods. Children with Burkitt’s lymphoma, less than 18 years and assigned to LMB96 protocol at CCHE. Their data between 7/2007 till end of 10/2015 were

retrospectively collected. They all had a second CVP after a pre-induction phase due to: 1-Renal failure, or creatinine clearance 10 folds. An initial full laboratory workup, whole body

CT scan, PET scan, bilateral bone marrow aspirate and biopsies and a CSF analysis were all done for all cases as well as an initial baseline cardiogram. A biopsy, will is obtained from

all patients.

Results. 45 patients received 2nd CVP 29 males and 16 females The site of primary disease was abdominal in 34 patients, pelvic in 5, iliac bone in one, ovarian in one, lumbar mass

in one, bilateral renal mass in one, paraspinal in one, and maxillary sinus in one. Nineteen patients were stage IV, while 26 patients were stage III. Twenty fi ve patients were group C (55.5 %),

and 20 were group B (44.5 %). After the 1st cycle of CVP, 8 had stationary disease, 34 had Regressive disease while 3 showed progressive disease. A second CVP was given due to renal

impairment in 3 patients, 3rd space fl uid accumulation in 17 patients, 6 patients had convulsions, one patient had encephalitis, while 4 patients had very poor general condition. After

a follow up period ranged from 17 days to 8.083 years, 29 were alive (64.5 %), while 16 patients (35.5 %) died (7 died immediately post 2nd CVP). Five patients died from disease

progression, 10 patients died from septicemia, one from multisystem organ failure. The period until patients had their Induction phase (COPADM-1) ranged from 6–45 days.

Conclusion. In mature B cell lymphoma of children and adolescence a second CVP is tolerated in ill patients but with a high price (35.5 % death) An alternative approach for those

patient who cannot tolerate induction should be searched for.

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A B S T R A C T N O . : P P - 2 3 4

Еvaluation of morbidity, mortality and survival in children and young adolescents

with malignant lymphomas in Arkhangelsk region in 2003–2014

N.A. Grigoreva

Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsov, Russia

Key words: malignant lymphomas, incidence, mortality, children, non-Hodgkin’s lymphomas, Hodgkin’s lymphomas, 5-year survival

Introduction. The incidence of malignant lymphomas (ML) still stays one of the fi rst places, gives way only for leukemias and brain tumors. According to US authors annually about

55 % of all ML are non-Hodgkin’s lymphomas (NHL). 5-year survival rate in Hodgkin’s lymphoma (HL) is nearly 95 % when this disease was detected early, and it is nearly 90 % after

late diagnosis. 5-year event-free survival in NHL is 80 %; this index is up to 90 % when NHL was detected early and in localized forms of NHL. Analysis of the incidence and survival

of the patients with ML is highly relevant in modern society from the standpoint of pharmacoeconomics, demography, improving the quality of life of the patients.

Aim. To evaluate the incidence, mortality and 5-year survival of patients with ML in Arkhangelsk region in 2003–2014.

Materials and methods. The analysis was performed on the database of medical records of Arkhangelsk Children’s Hospital, of Cancer Registry of Arkhangelsk Regional Clinical

Oncology Center.

Results. During 12-years period total were 52 patients with ML. From them 26 had NHL (50 %) and 26 had LH (50 %). Age of primary diagnosis of NHL was from 2.5 to 17 years old

(a median is 11.5 years old), age of primary diagnosis of LH was from 4 to 17 years old (a median was 15 years). Among patients with LH there were 4 children with I (15.4 %),

13 – with II (50 %), 4 – with III stage (15.4 %) and 5 – with IV stage (19.2 %). Among patients with NHL there were 2 children with II stage (7.7 %), 9 – with III stage (34.6 %) and

15 patients with IV stage (57.7 %). Primary incidence of all ML was maximal in 2003 (3.56 per 100 000) and minimal in 2008 (0,82 per 100 000), annual incidence of malignant

lymphomas was 1.79 ± 0.21 per 100 000. Primary incidence of NHL was 1.8 per 100 000 in 2013 (maximal) and 0.0 in 2011; primary incidence of LH was 2.1 per 100 000 in 2009

(maximal) and 0.0 in 2004 and 2013. Maximal level of mortality in children with ML occurred in 2003 and amounted to 2.22 per 100 000 children; the lowest level of mortality in

children with ML was in 2007 (0.40 per 100 000). In 2006, 2009–2014 we did not registered deaths among children with ML. 5-year survival (by Kaplan–Meyer) according nosology

was: among patients with NHL 68.7 %; among patients with LH 91.8 %.

Conclusion. In the structure of ML in 2003–2014 there were NHL and LH 50 % of both. The average annual incidence of malignant lymphomas was 1.79 ± 0.21 per 100 000,

this is slightly higher than European and US data, but comparable with Russian data. The median of age for NHL was 11 years, for LH it was 15 years, which is very typical for these

kinds of diseases. We have found that 50 % of LH were detected in the II stage of disease. Among NHL the majority (92.3 %) of cases manifested in the III and the IV stages, and 5-year

survival in LH are comparable to those in developed countries and Russian Federation. However, survival in NHL was lower (68.7 %). This was probably due to these patients were

admitted to oncology departments with advanced stages of the disease, often they were in terminal condition, sometimes they were admitted after non-correct surgical interventions

or too late. But in recent years there was detected decreasing of mortality in patients with malignant lymphomas, which can indirectly indicate positive experiences of a program

of a therapy of malignant lymphomas and the appearance of cancer alertness among pediatricians and other related specialists.

A B S T R A C T N O . : P - 2 4 2

Use of blinatumomab for treatment of early bone marrow relapse

of B-cell lymphoblastic lymphoma

D.A. Evstratov1, N.V. Myakova1, O.P. Khlebnikova2 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Children’s Regional Clinical Hospital № 1, Yekaterinburg, Russia

Key words: lymphoblastic lymphoma, relapse, blinatumomab

Introduction. Blinatumomab belongs to a class of recombinant bi-specifi c T-cell engagers (BiTEs). It is single polypeptide chain, containing binding sequence for the pan-B-cell

antigene CD19 on the one hand and for CD3 ε-chain of T-cell receptor on the other. BiTEs were developed for directing eff ector T cells to target cells with the activation of cytotoxic

T-lymphocytes. Blinatumomab targets CD19 that is expressed on acute lymphoblastic leukaemia (ALL) cells and on lymphoblastic lymphoma cells. Its action results in T cell-mediated

killing of blast cells. To date, numerous studies of blinatumomab for the treatment of refractory ALL, including in children, were published.

Aim. We present a case of blinatumomab use in a child with relapsed precursor B-cell lymphoblastic lymphoma.

Materials and methods. Our patient is an 11-year-old boy. The diagnosis “B-cell lymphoblastic lymphoma” was established in September 2012 based on the biopsy samples

of solitary soft tissue neoplasm located in the parietal region. No treatment was performed. Enlargement of lymph nodes occurred in December 2012. In March 2012 the diagnosis

“Stage II B-cell lymphoblastic lymphoma with the involvement of cervical lymph nodes” was made and treatment according to the Euro-LB 2002 protocol was initiated. Three months

after the end of supportive therapy (July 2015) the child started to experience intensive pain in the lumbar region, leukocytosis in blood of 20,000/mcl, blast cells – 1 %. Myelogram

showed total infi ltration by malignant blast cells with the phenotype CD19+/CD22cyt+/CD79a+/CD13+/CD58+/CD45+/CD56+. Cytogenetic investigation revealed trisomy 3 intact

cerebrospinal fl uid.

The child started to undergo treatment according to the ALL-REZ-BFM-2002 protocol in the group S3. During prophase leukocytosis continued to increase. After blocks F1-F2,

a myelogram showed 2 % of blast cells, according to the fi ndings of fl ow cytometry, minimal residual disease (MRD) was 1.421 %. Treatment according to the II-Ida protocol was

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carried out, after which blast cell count in myelogram was 10 %, MRD – 11 %. Thus, refractory course of disease was established. Starting from 20.11.2015 block R1 was carried out,

after which a myelogram performed at points showed 3.5–3 % of blast cells, some of them had signs of anaplasia, MRD was 1.02 %. Considering high MRD in the patient with early

relapse, it was decided to conduct blinatumomab treatment. On 16.12.2015 a blinatumomab course was initiated: Days 1–7 in a dose of 9 mcg/day, from Day 8 to 28 – a dose of

28 mcg/day continuous infusion. One hour before the infusion and prior to up-titration, dexamethasone in a dose of 20 mg was administered. On Day 2 of therapy a fever that was

scarcely lowered by paracetamol, it was lowered only on the following day. No other toxicity was registered.

Results. Before the start of blinatumomab course, CD19+ cell count in the peripheral blood had amounted to 0.0016 × 109/l, after the therapy, CD19+ cells were not detected.

The level of immunoglobulin G decreased from 8 g/l before blinatumomab treatment to 4 g/l afterwards. Myelogram performed after the end of blinatumomab course demonstrated

lowered bone marrow cellularity, bone marrow of polymorphic composition, blast cell count less than 5 %. According to the results of MRD, malignant blast cell population was not

found. The patient was indicated to undergo hematopoietic stem cell transplantation (HSCT).

Conclusion. The feature of this case, despite common classifi cation category “ALL and lymphoblastic lymphoma”, is that these nosological forms diff er according to incidence, age,

prognostic factors, treatment protocols and sensitivity to treatment. Relapses of lymphoblastic lymphoma have generally worse prognosis. Use of blinatumomab can help to overcome

therapy resistance and make a patient ready for HSCT in remission.

A B S T R A C T N O . : P - 2 4 9

The use of Kinesio Taping according to the lymphatic correction technique

in children in oncohematological practice

D.D. Shcheglova, P.M. Gorbylev

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia

Key words: children, taping, rehabilitation, edema, ultrasound investigation, computed tomography, magnetic resonance tomography

Introduction. Lymphatic edema is one of the most frequent complications in children with oncohematological diseases. It causes the delay of recovery process and deterioration

of the quality of life. In order to ensure speedy recovery and the best outcome of the main treatment it should be eliminated at the earliest stages of its manifestation. A modern

approach in functional diagnosis gives reason to the development of a new complex of rehabilitation taking into account not only vascular system disorders but also the disorders

of muscular and skeletal tissue that in its turn will provide a more objective monitoring assessment of the eff ectiveness of the current and planned rehabilitation actions.

Aim. To determine diagnostically signifi cant criteria of disorder of the pathology of the vascular system development and to develop methods of rehabilitation treatment in children

with hematologic and oncological diseases by means of reducing the edema and the intensity of side-eff ects and complications.

Materials and methods. Ultrasound diagnosis of lymphatic malformations, magnetic resonance diagnosis (MRI) of lymphatic malformations, computed tomography (CT) with

contrast enhancement of lymphatic malformations. Patients of this investigation are children undergoing rehabilitation treatment at the Treatment and Rehabilitation Scientifi c

Centre “Russkoe Pole” of the FSBI “FRC PHOI named after Dmitry Rogachev” of the Ministry of Health of Russia.

Results. After the performed work clinical recommendations for rehabilitation treatment with due regard to the obtained data concerning lymphatic correction at all stages

of treatment and children’s rehabilitation will be developed. Moreover the rehabilitation treatment will be carried out during the intensive treatment. Rehabilitation, oncology

and hematology, pediatrics can be potential areas of application.

Conclusion. The use of Kinesio Taping according to the lymphatic correction technique by means of reducing the edema will improve the patient’s general state of health and increase

physical activity, thus it will accelerate the pace of physical rehabilitation; reduce side-eff ects and complications after the intensive treatment; decrease the intensity of postoperative

soft tissue edema as well as improve microcirculation and faster acceptance of locally advanced fl aps that is especially important in children who underwent reconstructive plastic

surgery on the face and neck.

A B S T R A C T N O . : O P - 2 6 7

A study of the demographic profi le, type and treatment outcomes in paediatric

non-Hodgkin’s lymphoma at a tertiary care centre in Karnataka, South India

Arun Mundakakulathu Thomas, Appaji L, Arunakumari BS, Padma M, Avinash

T, Madhumathi DS, Rekha V Kumar, Prasanna Kumari

Kidwai Memorial Institute of Oncology

Key words: lymphoma, NHL, India

Introduction. Non-Hodgkin’s lymphoma (NHL) is the fi fth most common cancer in children under 15 years of age and it accounts for about 7 percent of the childhood cancers in the

developed world. Most Paediatric NHL’s are of high grade and have an aggressive clinical behaviour. There is a lack of published literature on the frequency, distribution and survival

of Paediatric and Adolescent NHL in India.

Aim. The aim of this study was to elucidate the demographic profi le, histological subtype and outcome of treatment in the department of Paediatric Oncology, Kidwai Memorial

Institute of Oncology (KMIO), Regional Cancer Centre for Treatment, Research and Rehabilitation, Bangalore.

Materials and methods. A systematic review was done of a total of 124 children between ages 0–15 years diagnosed with NHL at our centre from January 2009 to December 2013

in the Department of Paediatric Oncology. The information collated were the demographic profi le, duration of symptoms, histological subtype of the disease, treatment and outcome.

Results. Out of the 124 children with malignant NHL seen at KMIO, the median age was 7 years. The majority 86 (69.4 %) were boys and 38 (30.6 %) were girls, with a sex ratio of

2.26:1. The mean duration of symptoms was 7 weeks. The most common subtype of NHL was lymphoblastic lymphoma 49 (39.5 %) followed by Burkitt’s lymphoma 44 (35.5 %), ALCL

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16 (12.9 %), DLBCL 14 (11.3 %) and plasmablastic lymphoma 1 (0.8 %). The most common site was the Abdomen 43 (34.6 %), followed by Mediastinum 37 (30 %), lymph nodes and

Extranodal sites like brain, liver, nasopharynx and spine. Most of the patients, 96 (77.4 %) were in St Jude Stage III at presentation. A total of 89 children (71.8 %) opted to receive

treatment at our institute. The LLs were treated with ALL protocol (MCP841). Stage I and II Burkitt’s lymphoma and DLBCL were treated with COMP whereas Stage III and IV Burkitt’s,

DLBCL and Stage I – IV ALCL were treated with MCP 842 protocol chemotherapy. The mean survival time of the children treated was 71.28 ± 2.99 months.

Conclusion. NHL in children are aggressive but are treatable with excellent outcomes with good supportive care and protocol based therapy.

A B S T R A C T N O . : O - 2 9 0

Clinical profi le and prognostic factors in children with sporadic Burkitt’s lymphoma:

An Indian experience

Smitha HV, Nita Radhakrishnan, Veronique Dinand, Anupam Sachdeva

Sir Ganga Ram Hospital, New Delhi, India

Key words: Burkitt lymphoma, survival rates

Introduction. Burkitt’s lymphoma (BL) is a high-grade non-Hodgkin’s lymphoma accounting for 30–50 % of all paediatric lymphomas. The event-free survival of advanced

BL is 80–90 % in the developed world. There are published Indian literature which looks at disease frequency and clinical presentation of BL but none reports survival.

Aim. The aim was to analyze the clinical profi le, prognostic factors and survival rates in Indian children with BL.

Materials and methods. A retrospective analysis of BL was carried out from April 2009 to June 2015. Data retrieved from old records included age at diagnosis, sex, primary tumor

site, tumor stage, mode of diagnosis, site of metastases, blood counts, LDH, uric acid and outcome.

Results. There were a total of 18 patients. Median age at diagnosis was 5.5 years (3–14 years) with male preponderance (78 %). Thirteen patients had stage IV and fi ve had stage

III disease. CNS involvement was observed in 8 patients. None of our patients had an early stage. Most common primary site was abdomen (16 cases, 88 %) and other two had

Bell’s palsy. Common misdiagnosis was hepatic abscess (2 cases), Bell’s palsy (2 cases), intussuception and tuberculosis in one case each resulting in signifi cant delay in initiation

of treatment (4–12 weeks). TLS was observed in 13 patients (72 %) and eight (45 %) of them had renal failure at diagnosis. Hyperuricemia and high LDH of > 500 were seen

in 66 % and 70 % cases respectively. Median follow up was 35 months. Eight patients died (7 toxic deaths and the other CNS relapse). The 5 year OS and EFS for whole cohort was

50 % and 47 % respectively. Signifi cant diff erence was noted in OS and EFS between stage III (80 %, 60 %) and stage IV disease (40 %, 40 %). No signifi cant association was noted

with respect to nutritional status, uric acid, LDH, initial total leucocyte count or platelet counts and renal failure at presentation with outcome.

Conclusion. With availability of best supportive care survival rates for Pre B ALL in our center is 80 %. The same is not refl ected in BL unlike developed world which could be attributed

to advanced stage at diagnosis, renal failure, misdiagnosis and toxic deaths.

A B S T R A C T N O . : O P - 3 0 2

Strategy of treatment children with relapse/refractory Hodgkin’s lymphoma

N.S. Kulichkina, E.S. Belyaeva, G.L. Mentkevich, A.V. Popa

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: Hodgkin’s lymphoma, relapse, refractory, treatment results, autologic hematopoetic stem cells transplantation

Introduction. The modern programs for treatment children with newly diagnosed Hodgkin’s lymphoma (HL) allow achieve 10-years event free survival (EFS) and disease free

survival (DFS) 85–93 %, but 7–10 % of patients develop relapse and 2–5 % are initially refractory to standard chemotherapy even it was enough intensive. In Russian Federation

has never been any program for treatment patients with relapse and refractory HL so children got nonprogram therapy based on OPPA, COPP, ABVD, IVA, MINE which sometimes were

successful and some pts could achieve good response, but there were indefi nite number of courses. And as a result, the effi cacy of treatment is hampered by serious long-term adverse

eff ects, including heart and lung disease, and secondary malignancies.

Aim. To create the program chemotherapy for children with relapse/refractory HL and to prove the eff ectiveness of the therapy.

Materials and methods. From July 2003 to August 2015 35 children with relapse 18 (51.4 %) and refractory – 17 (49.6 %) were enrolled to the protocol. Average 3.5–18 years

(13.5 ± 4.2), males 22 (62.9 %) and female 13 (37.1 %). From 2003 to 2007 year all the patient have got two courses of ICE (Ifosfamide, Carboplatin, Etoposide) followed by auto-HSCT.

After getting preliminary results we made a decision to continue treating late relapsed patients by that therapy but refractory children by 4 courses ViGePP (Vinblastine, Gemcitabine,

Procarbasine, Prednisone) followed by auto-HSCT. Thus, therapy based on ICE got 14 (40 %) pts and ViGePP – 14 (40 %), 6 (17.1 %) children began getting ICE, but they did not

response and therapy was shifted on ViGePP, and one (2.9 %) got two courses of ViGePP with stable disease and the therapy was shifted on two additional courses of ICE.

Results. Complete response (CR) was in 20 (57.1 %) patients, partial response (PR) – 14 (40 %), progressive disease (PD) – one (2.9 %). All patients fi ve-year DFS 69.6 ± 82 %,

median follow up 95.7 ± 10.4 mo, EFS – 67.2 ± 9.4 %, median follow up 101.7 ± 9.9 mo. DFS for group of pts got the therapy based on 4 courses of ViGePP was better – 82.5 ± 11.5 %

then based on 2 courses of ICE – 56,5 ± 12.6 % and patients whose therapy was shifted on too – 57.1 ± 18.7 % (P = 0,13). DFS for children with refractory and treated by ViGePP was

also better comparing with group treated by ICE: 77.1 ± 14.4 %, n = 10 and 25.0 ± 21.7, n = 4 (P = 0,047). The most important was response on therapy: DFS in patients with CR

was 88.2 ± 8.0 %, n = 20, and 43.8 ± 13.5 %, n = 15 who did not achieve CR (P = 0.002). The same trend was checked in patient underwent by auto-HSCT (29 pts) and had achieved

CR before auto-HSCT then patients with PR: 84.8 ± 10 %, n = 16 and 44 ± 14.1, n = 13 (P = 0.012). Auto-HSCT was not made for 6 children – one died from disease progression,

two pts with early relapse had got a heart failure and lungs fi brosis, one with early relapse but achieved CR after the 2nd ViGePP – refused from the procedure and two had got late

relapse and CR after the 2nd ViGePP. Five children who did not get auto-HSCT but achieved CR are in complete remission and alive (median follow up 4 years).

Conclusion. Thus, children with relapse and refractory HL are curable by the program intensive chemotherapy. The most signifi cant factors for survival were CR achievement and use

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induction treatment regime consisted on 4 courses of ViGePP. Regime based on ViGePP can be suggested as a standard induction therapy for relapse/refractory HL. Chemotherapy can

be shifted on diff erent regime if the previous one has been failed.

A B S T R A C T N O . : P P - 3 8 1

Venous thromboembolism in children and adolescents with non-Hodgkin’s lymphoma

I. Begun, R. Tarasevich, I. Papkevich

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: venous thromboembolism, non-Hodgkin’s lymphoma, ultrasonography

Introduction. Thrombotic complications – the actual problem of pediatric oncology. During the last 10 years was established the rising incidence of diagnosis of venous

thromboembolism (VTE) in children with malignancies, which is consistent with global trends. The method of choice for the diagnosis of VTE is the Ultrasonography.

This is an aff ordable, non-invasive and informative method of diagnosis of this pathology in pediatric cancer patients.

Aim. To analyze the frequency and semiotics of defeat of symptomatic thrombotic complications in children with NHL during treatment of the underlying disease.

Materials and methods. An retrospective analysis of cases of venous thrombosis in 3733 children and adolescents with malignancies was made. All of patients received treatment

in our centre from 2001 to 2014. In the protocols ultrasound and medical histories found 128 patients with fi rst diagnosed venous trombosis aged 1–21 years (median 13 years) for

both sexes. The data were processed from 234 patients with NHL too. From they were chosen who had of clinical and ultrasound symptoms of the presence of thrombotic masses

in the lumen of the main veins.

Results. Venous thrombosis have been installed in 17 patients with NHL aged 4–18 years (median 15 years). From morphological variants most widespread was T-lymphoblastic

lymphoma (40 %). From mediastinal localizations predominated (65 %). In two cases on prehospital the VTE was as one of the fi rst disease manifestation. Trombotic masses were

located in the subclavian and/or internal jugular veins (76 %). The remaining cases constituted VTE in system of the great main veins of the arm, popliteal/femoral and iliac veins.

Complete occlusion of the venous vessels was observed in 82 % of cases. In 40 % of cases of thrombosis localization coincided with the localization of central venous lines (CVL) in

the subclavian and/or internal jugular veins. None of the patients had of pulmonary embolism. When analyzing the frequency characteristics was revealed that venous thrombosis

by ultrasound diagnosed in 7,3 % (0–24 %) of patients with NHL per year from such who the fi rst time during of the year were receiving treatment in our Centre. A comparative

analysis of similar data for 2001/07 and 2008/14 showed an increase in the frequency of diagnoses of thrombotic complications (Chi-square test 4.4; P < 0.05) for patients with

NHL receiving treated in hospital in these periods. The average annual growth of detection of this complication for the last 7 years was 1.1 %. Such situation in the last time may be

linked with the growth of attention of clinicians to identify thrombotic complications, with the increasing in the number of catheterizations of central and peripheral veins and with

the improving of the quality of primary diagnosis of VTE, but no with increasing in the incidence of NHL.

Conclusion. Venous thrombosis in children with NHL ranks fi rst in frequency within the nosology group and in second place in absolute terms among all pediatric patients with

malignant tumors. Over the past 7 years was a signifi cant increase in VTE in patients with NHL. VTE in most cases localized in the internal jugular and/or subclavian vein in patient

were combined with primary mediastinal tumor localization. Massive mediastinal tumor and CVL in a pool of superior vena cava probably reinforce each other as the VTE risk factors.

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A B S T R A C T N O . : P - 1 6 6

Clinical case of verifi cation of the Langerhans cells histiocytosis diagnosis

Yu.V. Shatohin1, E.D. Teplyakova1, I.V. Snezhko1, E.I. Kuzub1, O. Shatokhina2

1Rostov State Medical University, Rostov-on-Don, Russia; 2Rostov Cancer Research Institute, Rostov-on-Don, Russia

Key words: clinical case, histiocytosis

Introduction. Histiocytosis are a heterogeneous group of diseases, whose pathologic substrate are the cells of histiocytic series which include: tissue macrophages, monocytes,

dendritic cells and their precursors.

Aim. Langerhans cells histiocytosis is a disease characterized by proliferation of cells of the macrophage phagocyte system in the skin, bones, bone marrow, liver, spleen, lungs, lymph

nodes and in other organs and tissues. How a variety of clinical symptoms in Langerhans cell histiocytosis. How a variety of clinical symptoms in Langerhans cell histiocytosis with

this pathology show clinical case.

Materials and methods. Patient N., 30 y.o., contacted the clinic with complaints of weakness, malaise, rashes on the scalp, face, armpits, groin and perianal area. He has been ill

since the spring of 2008 when he fi rst noticed scalp rash. The patient consulted a dermatologist in 4 months, his condition was considered as seborrheic dermatitis manifestation

and local treatment was prescribed. The therapy gave no results and in a month the rash spread to the skin of the back, armpits and groin; scrapings detected the spores of Mucor

fungi. Antifungal therapy was conducted with little positive eff ect. In 2009 the concilium in Skin and Venereal Diseases Dispensary regarded the patient’s condition as chronic benign

Hailey-Hailey pemphigus, and the patient had a course of treatment with a positive eff ect in the form of reduced hyperemia and skin elements moisture. However, after the cancellation

of prednisolone skin lesions began to progress again. In 2012 the patient was diagnosed diabetes insipidus and autoimmune thyroiditis with hypothyrosis and the patient regularly

took Minirin and Eutiroks. In 2013 the patient developed spontaneous pneumothorax. In March 2014 computer tomography revealed pneumosclerosis with bullous emphysema from

centrilobular to panlobular all over the lungs. Mediastinal lymphatic nodes were not enlarged.

By 2014 papular, pyodermic , seborrheic skin lesions were recorded on the chin. The biopsy of skin lesions was performed in September 2015. Histological and immunohistochemical

study revealed: CD68 – positive granular in some cells; S100 – positive in a signifi cant number of cells; Langerin – positive in a large proportion of cells; CD20 – positive in clusters

of the few B cells; CD3 – positive in the few T-cells; Кі67 – negative 2 times, which allowed to establish the diagnosis of Langerhans cell histiocytosis.

Results. At the time of his visit to the clinic the patient had the following manifestations of the disease: polymorphic skin rash (local seborrheic, infi ltrative-erosive, papular,

pyodermic) on the face, scalp and in skin folds (axillary, inguinal and perianal area); diabetes insipidus; hypothyrosis; hepatomegaly; multiple cystic lesions of lung tissue.

Conclusion. The above clinical case report shows little awareness of physicians of histiocytosis, which led to the late verifi cation of the diagnosis (the diagnosis was established

7 years later the clinical manifestation of the disease). Such late diagnostics undoubtedly led to the progression of the disease and multiple visceral lesions most of which

are irreversible.

HISTIOCYTOSIS

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A B S T R A C T N O . : O P - 1 8 3

Late diagnosis of Langerhans cell histiocytosis (LCH) in children

D. Evseev, I.I. Kalinina, T.Yu. Salimova, D.D. Baydildina, А.А. Maschan, М.А. Maschan

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: Langerhans cell histiocytosis, diffi culties of diagnosis, hepatic fi brosis, damage of intestines

Introduction. Langerhans cell histiocytosis (LCH) in children is extremely rare. The main clinical manifestations are rash, bone involvement , rare – the involvement of liver, spleen,

bone marrow, lungs.

Aim. To describe clinical cases of the late diagnosis of LCH due to rare clinical manifestations (damage of intestines, hepatic fi brosis).

Materials and methods. There were examined 2 clinical cases in which there were diffi culties in diagnosis of LCH.

Results. We present the description of the cases.

Patient № 1. A girl, the onset at the age of 4 months with the appearance of rash, a biopsy was not performed, rash reduced spontaneously. At the age of 6 months – fever, blood

in stool, a colonoscopy with a biospy were performed. Diagnosis: total erosive colitis. During histological investigation LCH (medium-sized cells with high nucleocytoplasmic ratio,

oval hyperchromic nuclei (“coff ee bean”) positive for CD1a and S100, CD68,) was detected. At the moment of the transferral to the FRC PHOI – rash, hepatosplenomegaly, anaemia,

thrombocytopenia. The child received therapy according to the LCH III protocol with a positive eff ect.

Patient № 2. A boy, the onset at the age of 2 years, started with fever, hepatomegaly (ultrasound investigation showed inhomogeneous cystic ectasia of segmental bile ducts,

a choledochous duct), the diagnosis of Caroli disease was made. The Kasai operation was performed: laparotomy, cholecystectomy, excision of the choledochous duct, applying

porto-jejunal anastomosis (a biopsy was not performed) without changes over time. During investigation before the liver transplantation the computed tomography of the lungs

showed multiple air-fi lled cystic cavities, in the form of fi brocystic transformation of the lung tissue. In order to verify the diagnosis the puncture biopsy of the liver was performed.

According to the histological investigation: periportal infi ltration with lymphocytes, with the presence of single eosinophilic leukocytes and a small accumulation of histiocytes

around one tract, positive for CD1а and Langerin. 2 courses of therapy (IC 1 and IC 2) were performed- without eff ect. At the moment the child is alive, the involvement of the lungs

and the liver are considered to be permanent LCH complications, symptomatic treatment is being carried out.

Conclusion. Due to the rare occurrence of LCH in children and diffi culties of its diagnosis without specifi c clinical picture the diagnosis is made late that may result in the unfavourable

outcome.

A B S T R A C T N O . : O P - 3 1 3

Histiocytic infl ammatory response as predictor of relapse

in hematological and lymphoid malignancy

Atish Narayanrao Bakane, Revathi R Raj, Ramya S Uppuluri, Divya S Subburaj, Sreejith R Kurup

Apollo Speciality Hospital, India

Key words: histiocytic infl ammatory response, retrospective observational study, xanthogranulomatous histiocytosis

Introduction. There have been a few case reports published in literature documenting histiocytic infl ammatory response in children with hematological and lymphoid malignancies.

We present a series of 2 cases wherein histiocytosis was found to precede relapse of leukemia and lymphoma.

Aim. To substantiate that histiocytic infl ammatory response may be a predictor of relapse of leukemia and lymphoma.

Materials and methods. This is a retrospective observational study of 2 children with hemato-lymphoid malignancies treated at Apollo Speciality Hospital, Chennai which

is a tertiary referral centre in India.

The fi rst child is an 8 year old male child who was diagnosed to have CALLA positive ALL in 2014 and received chemotherapy as per UKMRC ALL 2011 protocol. He achieved complete

remission at the end of induction and was continued on consolidation, delayed intensifi cation and maintenance chemotherapy. During cycle 3 of maintenance chemotherapy, he was

noted to have painful swelling of the right knee joint. Imaging revealed osteolytic lesion involving the lower end of femur. Biopsy with histopathology and immunohistochemistry

confi rmed Langerhans cell histiocytosis. He was accordingly started on Vinblastine and prednisolone. In view of persistent disease, he then received 2 cycles of cladribine and

cytarabine. After 2 cycles, PET CT revealed persistent lesion in the right femur. In view of fever, bone marrow was repeated which confi rmed isolated bone marrow relapse of ALL.

The second child is a 16 year old male who was diagnosed to have Stage 2 Hodgkin lymphoma at Bangladesh in 2008 and had received 6 cycles of ABVD before being referred to our

centre. Assessment showed persistent disease in few mediastinal lymph nodes. He was given involved fi eld radiotherapy and was on follow up. In 2013, in view of abdomen pain

and USG revealing multiple enlarged lymph nodes, laparoscopic excision biopsy was done. Histopathology confi rmed xanthogranulomatous histiocytosis. He received Celecoxib

and oral steroids after which he was in remission. In 2016, PET CT was repeated in view of fever and loss of appetite, which revealed multiple enlarged lymph nodes with signifi cant

FDG uptake. Biopsy, histopathology and IHC confi rmed T cell rich B cell lymphoma.

Results. The fi rst child was given reinduction chemotherapy followed by MEC in view of persistent disease. He remained refractory with progressive disease and ultimately succumbed

to his illness. The second child has been started on salvage chemotherapy at the time of submission of this abstract. We plan to consolidate with autograft once in remission

Conclusion. Children with hematological and lymphoid malignancies who develop histiocytic infl ammation during chemotherapy or when in remission, should be followed up

with a high index of suspicion for relapse. Early diagnosis of relapsed disease and institution of appropriate treatment for both histiocytosis and malignancy may improve outcomes

in these children.

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A B S T R A C T N O . : O P - 3 1 4

Incidence and clinical spectrum of drug resistant bacterial infections

and associated mortality in pediatric hemato-oncology:

experience from a tertiary care centre in South India

Atish Narayanrao Bakane, Revathi R Raj, Sreejith R Kurup, Ramya S Uppuluri, Divya S Subburaj

Apollo Speciality Hospital, India

Key words: drug resistant bacterial infections, hemato-oncological conditions, resistance to carbapenems

Introduction. There has been a well documented upsurge in the prevalence of drug resistant bacterial infections among children with hemato-oncological conditions undergoing

treatment. Knowledge with regards to the above can pave the way to better antibiotic stewardship and improved preventive strategies to minimize irrational use. We present data

on the incidence and clinical spectrum of bacterial infections among this group of children, patterns of resistance and mortality associated.

Aim. To describe Incidence and clinical spectrum of drug resistant bacterial infections and associated mortality in pediatric hemato-oncology.

Materials and methods. We conducted a retrospective, observational study from January 2014 to December 2015, with regards to the clinical spectrum, incidence and prevalence

of bacterial infections among children undergoing treatment for hemato-oncological conditions and haematopoeitic stem cell transplants at a tertiary care centre in South India.

We analyzed the incidence of multidrug resistant sepsis, defi ned as resistance to carbapenems and studied the correlation between multidrug resistant sepsis and morbidity

and mortality. Various interventions have been instituted in order to reduce sepsis and improve outcomes after discussion with the Hospital Infection Control (HIC) team.

Results. The total inpatients at our centre in the last 2 years in the department of pediatric hemato-oncology from January 2014 to December 2015 have been 1252. The total number

of culture positive sepsis in 2014 and 2015 amounts to 50. Among the children developing culture positive bacteremia, 11 were during AML induction chemotherapy, 14 were during

ALL chemotherapy, 23 Post HSCT with GVHD, 1 during OEPA chemotherapy for Hodgkin lymphoma and 1 during chemotherapy for metastatic Ewing sarcoma.

The organisms isolated included Gram negative bacilli including E. coli, Klebsiella, Pseudomonas in 24, CONS in 15 (which were found to be insignifi cant), Enterobacter 3, Alpha

hemolytic streptococci 2, Acinetobacter 3, Ralstonia 1, St. aureus 1, C. elegans 1.

Out of the 50 culture positive cases, carbapenem resistance was found in 15 cases (30 %). 2 cases were ESBL producers and sensitive to carbapenems. Mortality rate among children

with multidrug resistant bacteremia is 2/15 (13 %).

The above data analysis led to several discussions with the Department of Hospital Infection Control. Interventions initiated in early 2015 included studying CARBA-R PCR in all

patients admitted for HSCT and high dose chemotherapy. Positive CARBA R resulted in early initiation of Colistin. In children with Carbapenem resistant bacteremia, Colistin was added

early. Preventive strategies included use of neutropenic care, specifi c neutropenic diet, use of granulocytes early prior to end organ damage.

Conclusion. Although the total number of children with culture positive bacteremia has been limited to 50 in the last 2 years at our centre, 30 % of these have been organisms with

multidrug resistance, particularly to carbapenems. The most common organisms isolated are gram negative bacilli. Mortality due to drug resistant bacteria sepsis has been 13 %

at our centre. Analysis of this data with regards to prevalent institutional fl ora and drug resistance has resulted in institution of several preventive strategies. These include increased

care during neutropenic period and early institution of Colistin to reduce mortality and improve survival.

A B S T R A C T N O . : O - 3 4 3

Study of late eff ects and permanent consequences

of pediatric onset Langerhans cell histiocytosis

G.O. Bronin, R.B. Miroshkin, E.A. Bezunov, E.V. Mironova, E.V. Fisun, Yu.V. Pashko, B.M. Kurmanov,

E.A. Promyslova, L.V. Sidorenko, A.F. Karelin, V.N. Kasatkin, N.N. Volodin

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: l angerhans cell histiocytosis, permanent consequences, late eff ects, cognitive functions

Introduction. Langerhans cell histiocytosis (LCH) is a rare disease characterized by variable biological behavior and course ranging from rapid life-threatening to self-resolving forms.

Permanent consequences (PC) can complicate both multi-system and single system LCH. Pathogenesis of PC remains obscure in many cases.

Aim. The main objective was to evaluate clinical features and severity of permanent consequences among survivors of pediatric onset LCH.

Materials and methods. 25 LCH survivors of LCH aged from 5 to 17 were admitted to our Rehabilitation Center for special examination and rehabilitation program. The median

of surveillance from the time of onset was 8.5 years. We performed standard clinical check-up with main focus of axiology and clinical features of LCH and its PC. Large number

of biochemistry and endocrinology tests was done and neurological survey according to the Extended Disability Status Scale (EDSS) and International Cooperative Ataxia Rating Scale

(ICARS) was performed as well. We used a battery of Neuropsychology tests of CANTAB eclipse (Cambridge Cognition, UK) for the assessment of cognitive functions.

Results. In 10 cases diff erent PC were found. Orthopedic problems were revealed in 4 cases and demanded physical treatment. Neurodegenerative disease was proven both

by magnet resonance imaging and by neurological assessment in two boys. The intravenous immunoglobulin treatment was initiated in both cases. Correction of hormone substitution

therapy demanded in 5 patients. The main cognitive functions remained intact in most cases. We proved a good level of planning skills, mastering the strategy, spatial memory, often

ahead of age. Nevertheless, the results of this study allowed us to determine areas of concern in the cognitive functions of the respondents, in particular, a tendency to impulsiveness

and lack of working memory capacity. Both disorders were associated with the frontal cortex location.

Conclusion. The PC in survivors of LCH can be found with relatively high frequency and can aff ect their quality of life. Special examination and sometimes treatment is needed even

in long term follow-up time after the discontinuation of the main therapy.

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A B S T R A C T N O . : P P - 3 6 0

Langerhans cell histiocytosis (LCH) of the female genital tract:

A case report and review of the literature

Husam Farid Bashir, Tal A Dujovny, Hertzel Gabriel, Yoseph Horovitz

HaEmek Medical Center, Afula, Israel

Key words: Langerhans cell histiocytosis, vulvar, LCH

Introduction. Langerhans cell histiocytosis (LCH) of the female genital tract is rare, LCH involving the genital tract, including the vagina, cervix, uterus, ovaries, and vulva has been

reported in adults. Primary LCH of the vulva is rare and even rarer in children. Because of the small number of cases reported, there is no standard

recommendation for the management of genital LCH.

Aim. Case report and review of literature.

Materials and methods. Methods of treatment include topical steroids, oral steroids, topical nitrogen mustard, thalidomide, interferon, surgery (vulvectomy or local excision),

radiotherapy and chemotherapy (vincristine, vinblastine, methotrexate, 2-chlorodeoxyadenosine).

Results. We report a case of a 14 year old child with vulvar LCH. After initial evaluation, she was treated according to the LCH-IV protocol, STRATUM I, with vinblastine + prednisone.

We describe the clinical presentation, evaluation, and treatment of primary vulvar LCH along with review of the literature.

A B S T R A C T N O . : P - 3 8 0

Histiocitosis of cells de Langerhans. Series of cases in pediatric population.

National institute of Oncology and Radiobiology (INOR) 1980–2015

Migdalia Perez, Mariuska Forteza, Jose Alert, Jesus Reno

National institute of Oncology and Radiobiology of Cuba, Havana

Key words: Langerhans cell histiocytosis

Introduction. Langerhans cell histiocytosis is a rare disease with diff erent clinical and pathological presentation, is clinically divided into three groups: unifocal, multifocal unisystem,

and multifocal multisystem. This enigmatic entity’s etiopatogenia is not all right clarifi ed. As of the present moment, investigators’s bigger number presents that one is produced.

Alteration in the regulation of the immune system in these patients. The presence of aggregates of another immunogenicly active cells in injuries, the anomalies found in the swindle,

the decrease in linfocitos’s number T suppressing CD8+ and the increase in several citocinas’s synthesis (factor macrofagos stimulator and little granules, IL-1, IL-3, IL-8, IL-10, TNF α

and interferon) suggest a physiological answer exaggerated of the CL to one Identifi ed antigen or, on the contrary, an appropriate answer to abnormal appropriate signals of another

cells of the immune system.

Aim. To characterize the pediatric patients with diagnostic of Langerhans cell histiocytosis tried in the National Institute of Oncology (INOR) during the period 1980–2015.

Materials and methods. A descriptive and retrospective, pediatric-patients enrolled in the Institute Nacional of Oncología and Radiobiología (INOR) with diagnosis of Histiocitosis

of cells of Langerhans during the period understood among the year 1980 and the 2008 study came true. Our sign of study got constituted for 32 patients with equal age or minor

to 15 years registered in the INOR with diagnosis of Histiocitosis of cells of Langerhans and that they received the initial treatment in the INOR during the mentioned period of time.

The bibliographic revision of the theme in point came true. The data got from case histories themselves, the archives of morbid anatomy were consulted and correspondent data bases

of oncopediatria’s service of the INOR and of the Record Nacional of Cancer.

He manufactured a model for the anthology of primary data, created for the purpose (I Annex # 1); Where they included the related variables to: The patient’s general data, clinical,

diagnosis, treatment elements and tracking.

The secondary data obtained of the fi lling of the model got into a data base using the program ACCESS 2003 and they processed themselves utilizing the statistical system

SPSS 11,5 for WINDOWS.

Results. The masculine sex prevailed (65.6 %) with ages of smaller or similar to 2 years (40.6 %). the located bony pain Prevailed (84.4 %), the bony aff ectation (90.6 %)

and the located illness (84.4 %). the stratum Prevailed of diagnostic presumptive (65.6 %). The used treatment was the chemotherapy like unique and combined modality;

with the protocol of sequential oral chemotherapy. The survival free of progression and global survival to the 10 years of 73.1 % and 86.9 %, respectively.

Conclusion. Langerhans cell histiocytosis is an illness of systemic commitment, with diverse forms of clinical presentation, what makes it an entity of diffi cult diagnostic

and handling. In the analysis of survival, association was observed statistically signifi cant with regard to the age, the extension of the illness and the answer to the treatment.

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A B S T R A C T N O . : O - 0 8 2

Segmental chromosomal alterations have prognostic impact in primary and relapsed

neuroblastoma patients

A.E. Druy1, E.V. Shorikov1, G.A. Tsaur2, S. Tuponogov1, L.I. Saveliev3, L.G. Fechina1 1Children’s Regional Clinical Hospital № 1, Yekaterinburg, Russia; 2Research Institute for Medical Cell Technologies,

Yekaterinburg, Russia; 3Ural State Medical University, Yekaterinburg, Russia

Key words: copy number variations, prognosis, primary neuroblastoma, relapsed neuroblastoma

Introduction. Neuroblastoma is characterised by wide clinical heterogeneity caused by the genetic features of the tumor. Segmental chromosomal abnormalities or copy number

variations (CNVs) are common alterations in the neuroblastoma genome. At the same time the prognostic signifi cance of many CNVs remains unclear.

Aim. Investigation of spectrum, frequency and prognostic impact of CNVs in primary and recurrent neuroblastomas.

Materials and methods. 139 primary and 22 relapsed neuroblastoma samples were studied for CNVs using MLPA. All primary and 14 relapsed tumors were obtained during

core biopsy. In 8 cases of recurrence liquid biopsy of involved bone marrow has been performed. Prognostic signifi cance was estimated by overall (OS) and event-free survival (EFS)

with median of follow-up time 36 months (range 1–190 months).

Results. In 32 patients (23.0 %) 1p deletion was revealed and had negative prognostic impact (EFS 0.38 SE 0.09 vs. 0.63 SE 0.05, P = 0.010, OS 0.49 SE 0.09 vs. 0.71 SE 0.05,

P = 0.008). 17q gain was detected in 60 patients (43.2 %), EFS 0.51 SE 0.07 vs. 0.63 SE 0.06, P = 0.041, OS 0.51 SE 0.08 vs. 0.74 SE 0.06, P = 0.021. Trisomy 7 discovered in 12 patients

(8.6 %) decreased survival: EFS 0.41 SE 0.16 vs. 0.59 SE 0.05, P = 0.032; OS 0.46 SE 0.18 vs. 0.65 SE 0.05, P = 0.045. Aberrations listed above retained prognostic signifi cance in MYCN

non-amplifi ed patients.

2p23-24 gain below the MYCN amplifi cation (MNA) level was detected in 13 patients and had negative signifi cance in group of patients below 18 moths: both EFS and OS 0.56 SE 0.20

vs. 0.82 SE 0.06, P = 0.048 and 0.92 SE 0.04, P = 0.024.

9p deletion with haploinsuffi ciency of CDKN2A gene was revealed in 9 patients (6.5 %) and resulted in low OS 0.38 SE 0.17 vs. 0.65 SE 0.05, P = 0.032. MDM2 gene gain had negative

infl uence on EFS in favorable groups: in infants (0.55 SE 0.13 vs. 0.86 SE 0.06, P = 0.011), in patients with localized disease (0.61 SE 0.11 vs. 0.79 SE 0.06, P = 0.057) and in 4S patients

(0.20 SE 0.18 vs. 0.86 SE 0.13, P = 0.043).

In multivariate analysis of OS stage 4 (P = 0.042), MNA (P = 0.049) and 9p deletion (P = 0.041) demonstrated independent prognostic signifi cance.

Investigation of CNVs in relapsed neuroblastomas revealed appearance of new alterations in 9, stable spectrum of aberrations in 3 and lack of original CNVs in 5 cases. Patients

harboring new CNVs had signifi cantly worse outcome after the recurrence comparing with those who had identical or lack of CNVs in relapse: EFS 0.00, OS 0.14 SE 0.13 vs. both

0.73 SE 0.16, P = 0.014, P = 0.045.

Conclusion. Thus CNVs have prognostic signifi cance in primary and relapsed neuroblastomas. This fact encourages performing tissue or liquid biopsies in cases of the tumor recurrence.

SOLID NON BRAIN TUMOURS

NEUROBLASTOMA

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A B S T R A C T N O . : O P - 0 8 9

Stromal Collagen type XI alpha 1 COL11A1 expression in neuroblastoma

Chi Bao Bui1, Hieu Kim Huynh2, My Diem Vu2, Dinh Khai Truong1, Tan Son Vo2

1Children Hospital II, Ho Chi Minh City, Vietnam; 2Ho Chi Minh City University of Medicine and Pharmacy, Vietnam

Key words: neuroblastoma, high-risk, MYCN, chromatin remodeling

Introduction. In invasive carcinomas, the extracellular collagens are key players of tumor behavior and are subjected to continuous remodeling in tumor progression, recurrence

and poor outcome. The COL11A1 human gene codes for the α1 chain of procollagen and mature-collagen 11A1, an extracellular component of ECM. It has been reported that high

expression COL11A1 participates in many cancers and is correlated with clinico-pathological features, suggesting its value as a useful prognostic factor for cancer patients. However,

such study is not yet investigated in Neuroblastoma, the most aggressive and heterogenous cancer in children.

Aim. The aim of this study is to clarify the COL11A1 expression and its relation with Neuroblastoma outcome in Vietnamese patients.

Materials and methods. A total of 80 NB patients in Children Hospital II were enrolled in this study. The expression levels of COL11A1 genes were measured using quantitative

reverse transcription polymerase chain reaction (qRT-PCR). Immunohistochemistry was used to localize COL11A1 expression in tumor or stroma region. Association with

clinoco-pathological NB and COL11A1 were assessed in specimens collected from primary, metastases and recurrent NB.

Results. COL11A1 expression was detected in 23 % of 83 NB and correlated with stage 3/4 (P = 0.23), recurrence (n = 8, P = 0.01), MYCN amplifi cation (P = 0.04). Immunostaining

showed that the COL11A1 co-localized with Vimentin in intratumoral stroma, but not the peripheral stroma. Remarkably, such COL11A1 mRNA expression is signifi cantly associated

with MYCN amplifi cation.

Conclusion. Our fi ndings examine the COL11A1 expression in advantaged cancer and suggest that microenvironment of stromal COL11A1 is a signifi cant risk for cancer prognosis

and disease progression.

A B S T R A C T N O . : P P - 0 9 5

Treatment of patients with neuroblastoma stage 4S

A. Kazantsev, P. Kerimov, M. Rubansky, O. Kapkova, M. Rubanskaya, D. Rybakova

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: neuroblastoma, 4S stage, risk group, survival

Introduction. In this study, we examine the prognostic risk factors in patients with neuroblastoma stage 4S, evaluate the results of treatment in this group.

Aim. To improve the survival of patients with stage 4S neuroblastoma.

Materials and methods. The study included 9 patients with neuroblastoma stage 4S, the average age of 5.6 (2–9) months. According to the classifi cation of Y. Shimada in

7 patients had a favorable histology, 1 patient – unfavorable and in 1 case study was not performed. DNA Index: 8 cases was the ploidy hyper detected in tumor cells, and only in 1 case –

hypoploidy. The most common sites of metastasis are liver (n = 8), and bone marrow (n = 7), 89 and 78 % respectively. 6 patients entered the intermediate-risk group, 3 patients –

at low risk. 7 patients received radical surgery. 1 patient at low risk and 6 patients in the intermediate-risk group received chemotherapy. No patient received radiation therapy.

Results. 7 patients had obtained a complete remission, 2 patients received a good partial response. Currently, all patients were alive with no evidence of recurrence. Overall survival

was 100 % with an average follow-up of 93.4 ± 18,7 months.

Conclusion. In the group of patients with neuroblastoma stage 4s can achieve excellent treatment results. Moreover, radiation therapy (which can lead to disability or development

of second tumors) is not necessary. For the same reason, you should avoid intensive chemotherapy, however, such an approach is possible with the correct stratifi cation of risk group

and consideration of all prognostic factors.

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A B S T R A C T N O . : P P - 1 0 9

Curative eff ect analysis of sequential autologous stem cell transplantation on high-risk

neuroblastoma in children

Jie Yan

Tianjin Medical University Cancer Institute and Hospital, China

Key words: sequential autologous stem cell transplantation, high-risk, neuroblastoma

Introduction. Nowadays, the prognosis of high-risk neurobalstoma is poor, although with comprehensive treatment of operation, chemotharepy, radiotharepy. We analyse

the outcomes of combining sequential autologous stem cell transplantation and traditional therapy.

Aim. To assess the outcomes of sequential autologous stem cell transplantation on high-risk neuroblastoma in children.

Materials and methods. There are 82 high-risk neuroblastoma between January 2010 and December 2014 in our hospital. Of which, 45 patients had accepted stratifi cation

treatment according to CCCG-NB09 Protocol, and 16 patients fi nished sequential autologous stem cell transplantation after chemotherapy and operation. There was 3 months interval

between two times ,we gave them radiotherapy. When two times transplantation fi nished, they accepted orally retinoic acid 6 months.12 patients fi nished two times sequential

autologous stem cell transplantation.

Results. The Kaplan–Meier curve revealed regular therapy according to CCCG-NB09 Protocol signifi cantly improve the prognosis of high-risk neuroblastoma. The median overall

survival time of 45 children who received regular therapy was 23.0 months, average survival was 25.1 months, the 1–4 year survival rate was 86.7 %, 70.6 %, 50.6 %, 40.6 %,

the response rate (CR+PR+SD) reaches upto 95.5 %.The side eff ects mainly were reversible bone marrow suppression and digestive tract reaction, not death relating to transplantation

and second cancer. We found 3-year total survival (59.7 %) after sequential autologous stem cell transplantation was superior to no transplantation (48.6 %). But the Log-rank test

showed there was no signifi cant signifi cance (P = 0.185).

Conclusion. Sequential autologous stem cell transplantation on high-risk neuroblastoma in children was superior to no transplantation, but the diff erence was not signifi cant.

So, we need to improve conditioning regimen.

A B S T R A C T N O . : O P - 1 2 2

Expression of CD133, CD24 in neuroblastoma and prognostic signifi cance

Qiang Zhao

Tianjin Medical University Cancer Institute and Hospital, China

Key words: CD133, CD24, neuroblastoma, prognosis

Introduction. Neuroblastoma (NB), which originates from the sympathoadrenal lineage of neural crest during the embryonic period, is one of the most common extracranial

malignant solid tumors in children. The characteristic features of NB are high degree malignancy, early multiple metastases, easy resistance to chemotherapy, and high rate

of recurrence. Stem cells is a kind of primitive cells that are: (1) self-renewing, (2) multipotent, and (3) diff erentiating. Recently, more and more evidence, suggests that very few

cancer stem cells exist in the tumor tissue, has been emerging. These cancer stem cells provide a reservoir of cells that can self-renew, maintain the tumor by generating diff erentiated

non-stem cells which make up the bulk of the tumor and are responsible for recurrence after surgery and chemoradiotherapy. A new theoretical basis has been provided for the

in-depth study of tumorigenesis and the evaluation of prognosis of cancer therapy. Also, new ideas which target killing cancer stem cells have been introduced for cancer therapy.

The aim of our study was to investigate the expression of CD133, CD24 in NB and also analyze the clinical and prognostic signifi cance, looking for new treatment targets of recurrent

and refractory NB.

Aim. To investigate the expression of CD133, CD24 in NB and its correlation with clinicopathological factors and prognosis.

Materials and methods. The expression of CD133, CD24 in 78 specimens of NB were assessed by immunohistochemistry.

Results. CD133 was expressed in 55.1 % (43/78) cases of NB, signifi cant association of its expression in NB tumor tissues with INSS Staging (P = 0.037), tumor diameter

(P = 0.040), pathologic types (P = 0.019) were found. CD24 was expressed in 60.3 % cases of NB, signifi cant association of its expression in NB tumor tissues with age (P = 0.008),

INSS Staging (P = 0.001), bone metastasis (P = 0.005), pathologic types (P = 0.038), preoperative chemotherapy (P = 0.027) were found. The survival time of CD133 negative patients

was signifi cantly longer than that of CD133 positive patients (P = 0.000). The survival time of CD24 negative patients was signifi cantly longer than that of CD24 positive patients

(P = 0.001). Age more than 1 year, recurrence and CD133 expression were independent prognostic factors in NB (P = 0.037, 0.001, 0.001, respectively).

Conclusion. Age more than 1 year, recurrence and CD133 expression were independent prognostic factors in NB, NB cells of CD133+ provide new potential targets for killing tumor stem cells.

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A B S T R A C T N O . : O P - 1 7 2

Incidence and management of chyle leaks following surgery

for abdominal neuroblastoma

Sajid S Qureshi, Monica Bhagat

Tata Memorial Hospital, Mumbai, India

Key words: neuroblastoma, chyle leak, abdominal surgery, conservative treatment

Introduction. Surgical intervention forms an important part of the therapeutic plan for neuroblastoma. However, surgery can be formidable considering the proclivity of these

tumors to encase important vascular structures, which makes the resection technically challenging, and often associated with complications. Chyle leak because of disruption

of retroperitoneal lymphatic channels is a possibility, however, its incidence and management after surgery for abdominal neuroblastoma is inadequately documented.

Aim. We analyzed our observations to fi nd risk factors and the optimal management of chyle leaks following resection of abdominal neuroblastoma. Second, we also evaluated

the impact of chyle leaks on further therapy, local control, and outcome.

Materials and methods. One hundred sixty patients who underwent surgery for abdominal neuroblastoma between September 2004 to August 2014 were evaluated. To fi nd

the oncological implication we evaluated the delay in starting further treatment, local control, event free and overall survival.

Results. Chyle leak was the most common complication (20 %). The median measure of leakage was 100 ml/day and it persisted for a median of 12 days. All patients were managed

conservatively except one who needed exploration for wound dehiscence. Number of lymph nodes resected was the only factor associated with the risk of chyle leaks (P = 0.013).

Adjuvant chemotherapy was not delayed in any patient because of chyle leaks per se and the local control, event free and overall survival was not diff erent for patients with and

without chyle leak.

Conclusion. Chylous leakage is a common postoperative complication of abdominal neuroblastoma, predisposed by the number of lymph nodes resected. It responds to conservative

management and does not compromise the further oncological treatment and outcome hence; it should not be a deterrent to complete surgery.

A B S T R A C T N O . : P P - 1 9 2

Characteristics and results of the treatment of patients with intermediate-risk

neuroblastoma in the Republic of Belarus

I.V. Praliaskouskaya, O.I. Bydanov

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: neuroblastoma, intermediate risk group, prognosis

Introduction. Until 2008 in RSRC DOHI the intermediate risk group included patients with neuroblastoma of stage 2B or 3, and patients under 1 year of age with stage 4 disease

were treated according to EINS protocol in which a high-dose polychemotherapy (PCT) with the support of autologous hematopoietic stem cell transplantation (auto-HSCT) was used

as a consolidating therapy. Since 2008 the intermediate risk group has included: patients with stage 2 or 3 disease with the presence of 1p deletion, patients over 2 years of age with

stage 3 disease, patients under 1 year of age with stage 4 disease (MYCN-negative) according to the NB-2004m protocol criteria. They receive treatment designed for the intermediate

risk group under the NB-2004m protocol, without auto-HSCT.

Aim. To estimate how changes in the stratifi cation of risk groups and therapy have infl uenced the outcomes of the treatment of intermediate risk group.

Materials and methods. One hundred forty protocol and observed patients who received treatment according to the NB-2004m protocol at the State Institution “RSRC DOHI”

from 2008 until 2016. A study group includes 22 patients of intermediate risk group. Fourteen (63.7 %) of them are boys and eight (36.3 %) are girls. Age distribution – from 4 days

to 7 years and 2 months, median age – 9.2 months.

Results. Patients from intermediate risk group amounted to 15.7 % (n = 22) of all the patients with neuroblastoma, patients from low risk group – 45.7 % (n = 65), patients from

high risk group – 40 % (n = 56). Distribution of patients according to stages was the following: stage 2 – 2 (9 %), stage 3 – 11 (50 %), stage 4 (children under 1 year of age) –

9 (41 %). Cytogenetic features: 1p deletion – 2 (9 %) patients, triploid tumours – 9 (41 %), di-tetraploid tumours – 13 (59 %). The best resection was the following: total resection –

6 (27.3 %), subtotal resection – 12 (54.5 %), tumour biopsy – 4 (18.2 %). An overall survival (OS) depending on a risk group was: 98 ± 2 % in the low risk group, 77 ± 9 %

in the intermediate risk group, 44 ± 10 % (P = 0.0001) in the high risk group. Event-free survival (EFS): 92 ± 4 % in the low risk group, 72 ± 10% in the intermediate risk group,

37 ± 8 % (P = 0.0001) in the high risk group. Cumulative recurrence rate was: 4.2 ± 2.9 % in the low risk group, 14.8 ± 8.1 % in the intermediate risk group, 59.1 ± 8.4 % (P = 0.001)

in the high risk group. Toxicity-related death was: 3.6 ± 2.6 % in the low risk group, 13.6 ± 7.5 % in the intermediate risk group, 2.2 ± 2.2 % (P = 0.065) in the high risk group. High

risk of mortality associated with treatment toxicity was registered in the intermediate risk group due to the death of 2 patients with stage 4 disease (children under 1 year of age)

caused by infectious toxicity and the death of 2 patients being under tumour induction. The comparison of the outcomes of the treatment according to the previously used protocols

for intermediate risk groups (stage 2B and stage 3 irrespective of patients’ age) including patients under 1 year of age with 4 stage disease and the outcomes of the intermediate risk

group treatment according to NB-2004m protocol has shown the following: OS in patients treated under the NB-2004m protocol – 77 ± 9 % vs previously used protocols – 78 ± 6 %

(P = 0.7910); EFS in patients treated under the NB-2004m protocol – 72 ± 10 % vs previously used protocols – 74 ± 6 % (P = 0.8391). The outcomes of the treatment of children

under 1 year of age with stage 4 according to EINS protocol used until 2008 and the outcomes of the therapeutic strategy used in NB-2004 group are the following: OS in patients

treated under the NB-2004m protocol – 68 ± 16 % vs EINS – 50 ± 20 % (P = 0.7742); EFS in patients treated under the NB-2004m protocol – 53 ± 17 % vs EINS – 50 ± 20 % (P = 0.9840).

Conclusion. The changes of intermediate risk group criteria through including a prognostically more unfavourable category of patients did not result in worse treatment outcomes of

the intermediate risk group. Patients under 1 year of age with stage 4 disease who receive treatment in this group do not require auto-HSCT but optimization of accompanying therapy.

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A B S T R A C T N O . : O P - 2 2 6

Outcomes of children with intermediate risk neuroblastoma:

Experience from a tertiary cancer centre in India

Monica Bhagat, Sajid S Qureshi

Tata Memorial Hospital, Mumbai, India

Key words: neuroblastoma survival, intermediate risk neuroblastoma, complete resection, gross total resection

Introduction. Patients with intermediate risk (IR) neuroblastoma generally have a favorable outcome; however, the intensity of treatment to achieve this outcome has been

debatable. In regards to the extent of surgical resection, confl icting results exist for both complete and incomplete resection.

Aim. To analyze the survival outcomes for patients with IR neuroblastoma and to study the impact of complete resection on the survival.

Materials and methods. Prospectively maintained database of patients who underwent surgery for neuroblastoma between January 2005 and January 2015 at a tertiary centre

was analyzed. Patients with Imaging Defi ned Risk Factors (IDRF) underwent biopsy prior to induction chemotherapy, while those with no IDRF were subjected to primary surgery.

he Kaplan–Meier method was used to compute the survival curves, while the log-rank test was used to analyze it.

Results. Of 229 patients with neuroblastoma who underwent treatment, including surgery, 75 patients had IR tumors. After a median follow up of 46 months, the 5-year overall

survival and event-free survival was 88.6 %, and 77.6 % respectively. There was no diff erence in survival between patients who underwent complete resection as compared

to those who underwent lesser degrees of resection (P = 0.789). There was no signifi cant diff erence in the incidence of intraoperative complications between patients who underwent

complete resection as compared to near complete resection (P = 0.638).

Conclusion. The extent of resection does not infl uence the survival of IR neuroblastoma, especially when multimodality therapy is the norm.

A B S T R A C T N O . : P P - 2 4 1

Neuroblastoma with asymptomatic epidural extension:

prolonged survival with palliative care

Issarang Nuchprayoon

Chulalongkorn University, Bangkok, Thailand

Key words: neuroblastoma, epidural involvement, long term follow up, natural history

Introduction. Neuroblastoma is often progressive with poor prognosis despite intensive treatment. However, neuroblastoma in infant may regress spontaneously. Epidural extension

of abdominal neuroblastoma often cause spinal cord compression and may need surgical intervention. There is few long-term information on asymptomatic intraspinal neuroblastoma.

Aim. We report here a case of abdominal neuroblastoma with extension into spinal canal but no cord compression symptom who elected for observation only and showed

no progression over 6 years.

Materials and methods. A 12-month old child presented with abdominal mass protuding through the right side of his back. He had a severe coarctation of aorta (COAT)

with ventricular septal defect and patent ductus arteriosus (PDA) which were diagnosed and had COAT repaired with division of PDA during early infancy. A biopsy of back mass reveal

neuroblastoma. He was given 14 courses of a chemotherapy regimen which included vincristine, doxorubicin, cyclophosphamide, & carboplatin every 3 weeks over a-12 month

period. A follow-up CT scan and an MRI of the abdomen at age 2 showed minimal response with large residual abdominal mass infi ltrating right iliopsoas and paraveterbral muscles,

circumferentially encasing aorta and inferior vena cava, with intraspinal extension through widened neural formamina of T12-L5. An MRI of the spine showed. There was lateral

displacement the thecal sac without spinal cord compression. At age 2, the child could walk for a few steps but no weakness nor incontinence. The child was referred to our hospital

for surgical intervention but the mass was determined to be unresectable and parents refused further chemotherapy as it impaired his quality of life and opted for palliative care.

He was followed up bi-annually by clinical examination only. Molecular details of neuroblastoma biopsied specimen will be available at the time of presentation.

Results. The child developed normally over the follow-up period of 6 years. Parents reported great quality of life without medical interventions. There was clinical signs of spinal

compression during the observation period. A complete MRI of the abdomen and spine at age 7 1/2 year revealed only slightly enlarged abdominal mass infi ltrating the right

paravertebral muscle with the same degree of intraspinal extension through widened neural foramina of T12-L5, without spinal cord compression.

Conclusion. Asymptomatic epidural extension of neuroblastoma may not need intervention. A watchful waiting approach is feasible.

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A B S T R A C T N O . : O P - 2 5 1

Treatment of high-risk neuroblastoma: experience of Russian federal centers

D.Yu. Kachanov1, T.V. Shamanskaya1, E.S. Andreev1, S.R. Talypov1, R.D. Khismatullina1, D.V. Shevtsov1,

E.V. Skorobogatova2, K.I. Kirgizov2, L.A. Hachatryan1, V.Yu. Roshchin1, Yu.V. Olshanskaya1, A.N. Kazakova1,

E.Yu. Osipova1, E.V. Raykina1, E.V. Feoktistova1, A.P. Shcherbakov1, G.V. Tereshchenko1, A.V. Nechesnyuk1,

N.S. Grachev1, D.K. Fomin3, M.A. Maschan1, Yu.N. Likar1, S.R. Varfolomeeva1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Children’s Clinical Hospital, Moscow, Russia; 3Russian Scientifi c Center of Roentgenoradiology, Moscow, Russia

Key words: neuroblastoma, high-risk, high-dose chemotherapy

Introduction. The prognosis of patients with high-risk neuroblastoma remains poor. The results of treatment of this group of patients in Russia are not fully studied.

Aim. The aim of the study was to analyze the results of therapy of high-risk neuroblastoma in three federal centers in Russia.

Materials and methods. 270 patients with NB were treated for the period 01.2012–06.2015 (42 months). The diagnosis has been established on the basis of the international

criteria (G. Brodeur, 1993). Patients were stratifi ed and treated according to the German NB-2004 protocol. 94 (34.8 %) patients were stratifi ed for the high-risk group. High-dose

preparative regimens included carboplatin/etoposide/melphalan (CEM) (till June 2013) and treosulfan/melphalan (TreoMel) (since July 2013). Since July 2014 patients with clear

MIBG-positive residual primary tumor and/or metastases prior to hematopoietic stem cell transplantation (HSCT) received 131I-MIBG-therapy.

Results. Male: female ratio was – 1.18:1. The median age at the diagnosis was 32.0 months (range 1.3–128.4). MYCN amplifi cation was observed in 43 (45.7 %) cases. 82

(87.2 %) patients had stage 4 NB. Induction therapy was completed in 90 (95.7 %) patients. 4 patients (all stage 4) didn’t complete the induction therapy (2 – progression, 2 – surgical

complications). Median number of chemotherapy cycles prior to transplantation was 6 (range 6–10). 78/90 (86.7 %) received high-dose chemotherapy: 28 – CEM, 50 – TreoMel.

Contraindications for HSCT included tumor progression (n = 7), organ toxicity (n = 3), surgical complications (n = 2). Transplant-related mortality was 3/78 (3.8 %), all in CEM group.

3-year EFS was 34.4 ± 6.3 % and 3-year OS – 46.6 ± 7.5 %. Stage signifi cantly predicted EFS (stage 1–3, 4S – 81.4 % versus stage 4 – 25.8 %, P = 0.007) in the high-risk NB patients.

In patients with stage 4 NB (n = 82) MYCN amplifi cation was observed in 31/82 (37.8 %), osteomedullary metastases in 73/82 (89.0 %) patients. Induction therapy was

completed in 78 (95.1 %). 9 (11.0 %) patients progressed during the induction. Complete, very good partial response and partial response were achieved in 68 (82.9 %) patients.

66/82 (80.5 %) patients received HSCT. 14/82 (17.0 %) with MIBG uptake after the induction received 131I-MIBG-therapy. Analyses of prognostic factors in stage 4 NB showed that

non-osteomedullary metastases (P = 0.01), MYCN amplifi cation (P = 0.03) and progression on the induction (P = 0.00006) were associated with inferior EFS in univariate analysis.

In the multivariate analysis, pattern of metastases and response to the induction were the most signifi cant variables associated with EFS. 3-year EFS and OS in the cohort of stage

4 patients was25.4 ± 6.8 % and 37.9 ± 8.5 %. Median time to relapse was 14.2 months (range 0.8–32.3 months). Most relapses were systemic (22, 55.0 %) or combined (16, 40.0 %).

Conclusion. Our results are consistent with other research groups. Intensive therapy allows achieving satisfactory results of therapy in high-risk NB patients with stages 1–3 and 4S.

The introduction of novel therapies are urgently required to improve prognosis in stage 4 NB.

A B S T R A C T N O . : P - 2 5 4

Epidural compression at the onset of neuroblastoma

D.Yu. Kachanov, T.V. Shamanskaya, O.B. Malevich, S.P. Khomyakova, S.S. Ozerov, E.S. Andreev,

G.V. Tereshchenko, Yu.N. Likar, S.R. Varfolomeeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: epidural compression, neuroblastoma, children

Introduction. Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Neurological symptoms as a result of epidural compression (EC) by tumor are frequent

initial presentation of NB.

Aim. To analyse the frequency, clinical presentation and therapeutic tactics in patients with NB and EC.

Materials and methods. The study included 18 children with NB during the period 01.2012–09.2015 (45 months). Diagnosis of EC has been established on the conclusion

of the neurological examination and neuroimaging data. All patient were assessed according to the standardized protocol and the diagnosis was established based on histological

examination and laboratory fi ndings.

Results. EC in the onset of the disease occurs in 18 of 277 (6.4 %) children with NB. The male: female ratio was 1:1.5. Median age was 8.5 month (range 0.5–85.3 months).

The most common topography of the primary tumor was retroperitoneal space in 12/18 (66.7 %) , posterior mediastinum in 6/18 (33.3 %) cases. EC was often found in patient with

stage 3 (6/18 – 33.3 %) and stage 4 (6/18 – 33.3 %) of disease. Stage 2 was observed in 4/18 (22.2 %) and stage 4S stage in 2/18 (11.2 %). Risk group distribution: low risk group –

1/18 (61.1 %), 5/18 (27.7 %) – intermediate risk group and 2/18 (11.2 %) – high risk group.

When was collected history of disease, has been established that 4/18 (22.2 %) patients before admission were not examined by a neurologist; unknown date of onset of the disease.

In these children EC was diagnosed at the initial examination by a neurologist in the case of after diagnosis of NB. For 14/18 (77.8 %) children time to onset of clinical symptoms till

to diagnosis EC was 2.6 months (range 0,4–5,1 months.).

In the study of neurological status in onset of the disease motor and sensitive dysfunction was found in 18/18 (100 %) cases. In 4/18 (23.5 %) children disease motor and sensitive

dysfunction combined with bladder and bowel dysfunction. For 8/18 (44.4 %) patients found disturbances in motor/sensitive sphere and radicular syndrome. Among motor disorders

in 3/18 patients (16.7 %) –monoparesis lower limb; у 1/18 (5.5 %) – tetraparesis; у 14/18 (77.8 %) – paraparesis lower limb.

Surgical treatment to resection the tumor from the spinal canal, performed 7/18 (40 %) patients (4 of them received additional chemotherapy). Chemotherapy according to risk-group

10/18 (55.5 %) patients. Surgery to resection only the tumor of the posterior mediastinum performed in 1/18 (5.5 %) patient.

For this time alive 16/18 (88.9 %) patients. Died of complications related to the treatment 2/18 (11.1 %) patients. The duration of follow-up was 24 months (range – 0.1–35.7 months).

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Neurological status after a specifi c therapy is presented in the form of improvement of motor functions in 5/16 (31.2 %) patients; in 11/16 (68.7 %) noted the preservation of paresis

to 4 score and bladder and bowel dysfunction.

Conclusion. In children with EC, to assess the degree of severity of the neurological manifestations needed work out and introduction into clinical practice a standardized scale,

perform of additional methods of examination, including in a multi-disciplinary group with neurosurgeon and rehabilitologist. Patient management tactics is determined taking the

duration and severity of symptoms EC, stage of disease and risk groups, the presence of the clinic experience of conducting such patients, the possibility of neurosurgical operations.

A B S T R A C T N O . : P P - 2 5 6

Retrospective comparison of treosulphan/melphalan versus

carboplatin/etoposide/melphalan as preparative regimen

for autologous transplantation in pediatric neuroblastoma

R.D. Khismatullina

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: high-risk neuroblastoma, autologous stem cell transplantation, preparative regimen

Introduction. High-dose chemotherapy with autologous stem cell transplantation is a standard component of multimodality treatment for high-risk neuroblastoma. Common

high-dose preparative regimens for neuroblastoma include busulphan/melphalan (Bu/Mel), carboplatin/etoposide/melphalan (CEM) and TBI-based combinations. Defi nitive

comparison of alternative preparative regimens has not been performed thus far.

Aim. In the current study we analyse retrospectively the results of autotransplantation with two regimens, CEM and treosulfan/melphalan (Treo/Mel), used consecutively for

conditioning in patients with high-risk neuroblastoma.

Materials and methods. Fifty six patients with high-risk neuroblastoma were treated in the Federal Center for pediatric hematology and Russian children’s hospital since April 2012

till September 2015. All patients were treated according to the NB-2004 protocol, excluding MIBG therapy, which was not available till July 2014. Nine patients were treated according

to the NB-2004 protocol, including MIBG therapy, since July 2014 till September 2015. But these patients are not included in this study. Two types of preparative regimens were

used. Twenty eight (f-7, m-21, median age 2.53 years) recieved CEM regimen (since September 2012 till June 2013): Melphalan 45 mg/m2/day, day -8,-7,-6,-5, Etoposide 40 mg/kg,

day -4, Carboplatin 500 mg/m2/day, day -4,-3,-2, G-CSF 10 mcg/kg/day IV since day +5 till WBC > 5 × 109/l. Twenty eight patients (f-14, m-14, median age 2.73 years) recieved

Treo/Mel regimen (since July 2013 till September 2015): Treosulfan 14 g/m2/day, day -5,-4,-3, Melphalan 140 mg/m2, day -1, G-CSF 10 mkg/kg/day since day +5 till WBC > 5 × 109/l.

The median dose of infused CD34+ cells was 10 (3.5–26) × 106/kg and 7.55 (2.7–31) × 106/kg respectively. The groups were balanced for disease stage, response to induction therapy

and bone marrow involvement. There was a trend towards overrepresentation of MYC-N amplifi cation (68 % vs. 46 %, P = 0,073) in the CEM group.

Results. Primary engraftment was achieved in 53 of 56 pts, the median time to neutrophil and platelet recovery was 10 and 15 days in the CEM group, 11 and 13.5 days in the Treo/Me

group, respectively. In the CEM group 3 pts died of bacterial sepsis before 100-day after SCT, 1y. pTRM – 10.6 % (95 % CI: 3.7–31.2). None of the patients died of transplant

complications in the Treo/Mel group, 1y. pTRM 0 %. In CEM group (median follow-up 3,2y) at 2 year cumulative incidence of relapse is 53.6 % (95 % CI: 38–73.8), pEFS is 35.7 %

(95 % CI: 18–53.5), pOS – 67.9 % (95 % CI: 50.6–85.2). In the Treo/Mel group (median follow-up 1.77y) 2 year cumulative incidence of relapse is 50.5 % (95 % CI: 34–75.1),

pEFS is 51.3 % (95 % CI: 30.9–71.7), pOS – 87.4 % (95 % CI: 73.9–100). There is a trend towards improved overall survival (P = 0,043) in the Treo/Mel group.

Conclusion. In this retrospective analysis Treo/Mel preparative regimen was associated with improved short-term results in comparison to the more conventional CEM regimen

in high-risk neuroblastoma. Further observation, matched pair analysis and prospective testing will be performed to confi rm these results and account for potential bias.

A B S T R A C T N O . : O P - 3 0 3

Мinimal invasive surgical treatment of children with thoracoabdominal localization

E.S. Andreev, T.V. Shamanskaya, M.N. Sukhov, D.Yu. Kachanov, N.G. Uskova, G.V. Tereshchenko,

N.S. Grachev, A.P. Shcherbakov, S.R. Talypov, S.R. Varfolomeeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: neuroblastoma, minimal invasive surgery, children, oncology, laparoscopy, thoracoscopy

Introduction. Minimal invasive surgical treatment is becoming more common in pediatric oncology. One of the perspective directions is a videoendosurgical treatment of children

with thoracoabdominally localized neuroblastoma (NB).

Aim. Surgical treatment improvement in children with NB of thoracoabdominal localization.

Materials and methods. From January, 2012 to January, 2016 (48 months) 197 patients received a surgical treatment in the extent of NB resection. Endosurgical operation was

undertaken in 54 (27.5 %) patients. All patients were observed and received a treatment according to NB-2004 protocol. For all patients a complex examination was undertaken,

surgical risks (IDRF) evaluation was carried out, therapeutic approach was accepted on a interdisciplinary discussion. Endosurgical treatment indication was: the absence of surgical

risks (IDRF), previous traumatizing surgical interventions, anatomically localized tumor of up to 7 cm in diameter.

Results. Median age was 20 months (range – 1–96 months), under 1 year – 26 (48 %) children. Stage distribution/allocation: 1st – 29 (54 %) patients, 2nd – 11 (20 %),

4th – 10 (18.5 %), 4S stage – 4 (7.5 %). Thoracoscopic tumor resection was performed in 14 (26 %) patients, laparoscopic tumoradrenalectomy – 36 (66.6 %), laparoscopic resection

of paravertebrally localized tumor – 4 (7.4 %). Tumor volume – 1 to 7 cm in diameter. Average surgery duration was 119 minutes. 2 (3.7 %) cases of hemorrhage were reported

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intraoperatively, that required a conversion and hemostasis, in 2 (3.7 %) cases the conversion was performed because of complications of tumor isolation. In 1 (1.8 %) female patient

an early postoperative period was complicated by sepsis, in 4 (7.4 %) patients Horner’s syndrome was reported after thorascopic tumourectomy. In 1 (1.8 %) patient adhesive

small bowels obstruction was developed, that required a second-look surgery. Early postoperative period proceeded faster and easier after minimal invasive treatment: early terms

of removing from an artifi cial lung ventilation, less expressed pain syndrome, early activation, cosmetic eff ect. Follow-up median was 16.2 months, local relapse was reported

in 1 (1.8 %), that required a second-look upfront surgery.

Conclusion. The minimal invasive surgery of children with thoracoabdominally localized NB, with contraindications and surgical risks (IDRF) absence, is highly eff ective method,

that allows to perform ales traumatic radical surgery with cosmetic eff ect and without oncological prognosis decrease.

A B S T R A C T N O . : O P - 3 0 5

Combination of chemotherapy and targeted therapy in treatment of very high-risk

patients with neuroblastoma and Ewing sarcoma family of tumors

I. Kazantsev1, A. Gevorgyan1, T. Jukhta1, A. Kozlov1, S. Shiriaev2, P. Tolkunova1, A. Klimov1, J. Rakhmanova1,

E. Morozova1, Yu. Punanov1, S. Safonova1, L. Zubarovskaya1, B. Afanasyev1

1Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia; 2N.N. Petrov Research Institute of Oncology, St-Petersburg, Russia

Key words: neuroblastoma, Ewing sarcoma, resistant and refractory, chemotherapy, targeted therapy

Introduction. Neuroblastoma (NB) is the most frequently seen pediatric extracranial tumor, while Ewing sarcoma family tumors (ESFT) are second most common bone malignancy.

Although the implication of intensive multimodal treatment programs allowed achieving an improvement in overall treatment results, there are still small therapy-resistant

subpopulations with dismal prognosis and very short life expectancy.

Aim. Investigating the eff ectiveness of chemotherapy combination with targeted agents in very high-risk and heavily pretreated patients with NB and ESFT.

Materials and methods. A total of 40 patients with NB (n = 32) or ESFT (n = 8) received therapy in our department in July 2009 – December 2015. All patients belonged to very

high-risk group due to primarily disseminated disease with multiple bone lesions (n = 29), primary resistance to 2 or more lines of chemotherapy (n = 25) including autologous

(n = 14) or allogeneic (n = 6) hematopoietic stem cell transplantation (HSCT), 1st resistant relapse (n = 9), 2nd or further relapse (n = 9). Most (35 of 40) patients previously had no

response to standard topoisomerase I inhibitors-based therapy. Most patients had progressive disease (n = 22), others had PR (n = 10) or stabilization (n = 6). Two patients received

preemptive therapy post-HSCT. Therapy consisted of sirolimus (1 mg/m2 × 4), dasatinib (2 mg/kg × 4) with consequent irinotecan (50 mg/m2 × 5) and temozolomide (150 mg/m2 × 5).

The median number of courses given was 3 (range 1–12). All patients with measurable lesions (n = 39) were restaged based on RECIST 1.1 criteria after each 2 therapy courses. For

NB patients with I-MIBG avid lesion (n = 23) 123I-MIBG scintigraphy was also used. Some patients with good response proceeded to high-dose consolidation with autologous (n = 7)

or allo-HSCT (n = 4).

Results. Therapy was eff ective in 32 of 40 patients (CR in 12.5 %, PR in 32.5 %, and stabilization in 32.5 % of cases), although the eff ect was mostly short lasting with a median

of 3 (2–48) months. Only 7 (17.5 %) of 40 patients are currently alive with a median follow-up of 10 (4–52) months. While in NB patients CR or good PR was observed in 19 of

32 (60 %) cases only 5 of 8 ESFT patients achieved a short-term stabilization. As expected, patients with progressive disease had worse (40 %) response rate, although in 2 cases

CR was observed. All patients with less aggressive disease responded for a median of 8 (2–48) months in spite of previous ineff ective exposure to topoisomerase I inhibitors. High-dose

consolidation in good responders (n = 11) was ineff ective, all but one patient relapsed in a median of 8 (4–39) months. One of post allo-HSCT preemptive therapy recipients died

of acute graft-versus-host disease, another is alive and stable 42 months after HSCT. The therapy regimen toxicity was relatively mild, even in HSCT recipients with hematological

toxicity critical for therapy timing developing in 4 and severe irinotecan-associated diarrhea in 6 cases.

Conclusion. Combination of irinotecan-based chemotherapy with sirolimus and dasatinib allows achieving response in very high-risk patients with NB in spite of previous resistance

to topoisomerase I inhibitors, although in resistant ESFT cases its advantage over standard therapy is not evident. However, results achieved are mostly short-termed in spite of dose-

intensive consolidation. The regimen toxicity is relatively mild; its low hematological toxicity allows employment in post-HSCT settings.

A B S T R A C T N O . : P P - 3 3 3

Vascular “Image-defi ned risk factors” in abdominal neuroblastoma

that were determined by ultrasound

I. Begun, I. Papkevich, R. Tarasevich

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: neuroblastoma, ultrasound, image-defi ned risk factors

Introduction. The most common sites of origin of neuroblastic tumors are the adrenal region and extraadrenal retroperitoneum (65–73 %). Clinical stage is currently

the most statistically signifi cant and clinically relevant prognostic factor. In parallel, can be used IDRFs – image-defi ned risk factors by Consensus Report from the International

Neuroblastoma Risk Group Project (2011). Stage L2 tumors by IDRFs are local-regional tumors with one or more IDRFs. While metastatic disease (stage M or MS) is staged oblivious

of the local-regional tumor extent, the absence or availability of an IDRFs for the primary tumor must always be assessed to aid surgical decisions.

Aim. The purpose of the study was to determine the frequency of vascular IDRFs in patients with abdominal neuroblastoma through capability ultrasound.

Materials and methods. Were studied result of ultrasound examination of 32 children aged from 0.1 to 10 years (mean 3.0 ± 2.5) with a prospectively established diagnosis of

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neuroblastoma of the adrenal region and extraadrenal retroperitoneum in stage 1–4, 4s (ISSN). Ultrasound data were verifi ed by comparing with the results of computer tomography.

Results. Mean tumor volume in patients amounted 350 ± 314 cm3. Abdominal aorta and it branches were involved in the tumors of the extraadrenal retroperitoneum in 82 %

cases and in the tumors of adrenal region – in 30 % cases. The anterior displacement of abdominal aorta from 3–4 mm till centimeters (mean 12 ± 8 mm) in 50 % patients with

the spread of the tumor under aorta was confi rmed. Tumor encasing origin of celiac axis and/or origin of superior mesenteric artery was in of 46 % all cases, tumor invading one or

both renal pedicles was in 38 % cases, tumor encasing aorta and/or vena cava was in 53 %. Simultaneous involvement of all of the above vessels was in 23 %. In one case we observed

the invasion of a tumor in the inferior vena cava. It has been found that the angle of divergence of the superior mesenteric artery and the aorta in patients with tumor of extraadrenal

retroperitoneum bigger than in the patients with tumors of adrenal region (P = 0.02). On the data of the Power Doppler and pulsed-wave Doppler were noted intratumorally

branching vessels, irregular diameter of the vessels, arteriovenous shunts in the tumor. In the cases with the calcifi cation of neuroblastoma the vascularization of tumor was a low.

In the remaining cases were determined by the average degree of tumor vascularization. The mean values of resistance index for vessels within the tumor was 0.64 ± 0.15.

Conclusion. The ultrasound duplex scan is the useful method in determining the involvement of major vessels of abdominal aorta in the retroperitoneal tumor. The ability

to accurately quantify to defi ne the indicators such as the anterior displacement of abdominal aorta and the angle of divergence of the superior mesenteric artery and the aorta

in patients with tumor of extra adrenal retroperitoneal increases the value of the method. The constitutional features of the childhood allow easy to select of the optimal acoustic

windows for ultrasound investigation to obtain precise contours of the vascular tree, which is particularly useful for the dynamic assessment of the tumor during chemotherapy.

A B S T R A C T N O . : P P - 3 7 1

Introduction the 131I-MIBG therapy in treatment of patients

with high risk group neuroblastoma in Russian Federation

D.Yu. Kachanov1, D.K. Fomin1, T.V. Shamanskaya1, D.V. Shevtsov1, G.M. Muftakhova1, Yu.N. Likar1, R.D. Khismatullina1,

M.A. Maschan1, E.V. Skorobogatova2, L.A. Khachatryan1, O. Barskiy3, G.A. Novichkova1, S.R. Varfolomeeva1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Children’s Clinical Hospital, Moscow, Russia; 3Innovative Treatment Systems Ltd

Key words: neuroblastoma, 131I-MIBG, childhood cancer

Introduction. The long term survival in high risk group patient’s with neuroblastoma (NB) is very poor, which become a reason to intense the therapy. The 131metaiodobenzylguanidine

(131I-MIBG) therapy is one of the options to improve treatment results in this patient’s group, after induction therapy.

Aim. To analyze indications for 131I-MIBG therapy application in group patient’s with NB within multicenter interaction.

Materials and methods. 14 patients with high risk group NB were included into our research. Observation period was from 07.2014 to 12.2015 (18 month). All the patients received

therapy according to NB-2004 protocol. The 131I-MIBG positive lesions (primary tumor/metastasis) which had been detected after induction therapy became the indication for using 131I-MIBG therapy. Quantitative evaluation lesions of pathological accumulation radiopharmaceuticals, was used during the metaiodobenzylguanidine (MIBG) scintigraphy (Curiе

score). The dose of radiopharmaceuticals was 12 mCi/kg during the 131I-MIBG therapy.

Results. The average age was 39.8 month at the primary diagnostic (from 18.4 to 64.4). Seven patients (50 %) had retroperitoneal primary tumor localization, 6 (42.9 %) – adrenal

glands, and in one (7.1 %) case in posterior mediastinum area. All the patients were diagnosed 4 stage of the main disease. Twelve patients (85.7 %) had the bone marrow metastasis,

11 (78.5 %) – in bones, 7 (50 %) – in lymph nodes, 1 (7.1 %) – in liver and 1 (7.1 %) in lungs. Bone marrow and/or bones were aff ected with metastasis in thirteen patients (92.8 %).

The MYCN amplifi cation was observed in 4 (28.5 %) cases.

Twelve patients (85.7 %) recived 131I-MIBG therapy after 6 courses of induction therapy, 2 (14.3 %) – after 8 courses. The median days from the last course of chemotherapy to 131I-MIBG

therapy was 30 days (from 22 to 42 days). MIBG positive lesions from primary tumor and metastasis were observed in 5 patients (35.7 %), 4 (28.6 %) had MIBG positive residual

tumor (without any accumulation in metastatic areas), and 5 patients (35.7 %) had MIBG positive only metastatic lesions (without accumulation in primary tumor area). The dose

of radiopharmaceuticals in all cases was strict - 12 mCi/kg. After 131I-MIBG therapy any complications were not observed. All the patients received autological stem cell transplantation

after 131I-MIBG therapy. The median from day of 131I-MIBG therapy to conditioning was 13 days (from 10 to 21 days). The median of engraftment was 11 days (from 9 to 16 days).

Veno-occlusive disease was not observed in any case. The average follow up period was 11.9 month (from 7.7 to 19.3). Twelve patients (85.8 %) have being survived without

any events, 1 patient (7.1 %) – have being survived after the progression of the disease, 1 (7.1 %) died after the progression of the main disease.

Conclusion. Our investigation demonstrated the ability to introduce the new treatment method for patients with high risk neuroblastoma, within multicenter and multidiscipline

cooperation in Russian Federation. The designed algorithm of prospective planning and patient’s selection for 131I-MIBG therapy allows to include the new method to the multimodal

therapy without infringement of timing. The long follow up period allows to evaluate the infl uence of therapy option into the main disease prognosis.

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A B S T R A C T N O . : P - 0 9 3

Results of treatment of children with bilateral nephroblastoma

M.A. Rubansky, A.P. Kazancev, P.A. Kerimov, M.V. Rubanskaya, D.V. Rybakova, A.V. Khizhnikov, O.A. Kapkova

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: treatment, results, nephroblastoma, children

Introduction. Wilms tumor is the most common renal cancer in children. Approximately 5 % of children with Wilms tumor present with disease in both kidneys. We retrospectively

reviewed our institutional experience of treatment patients with bilateral Wilms tumor (BWT) from 1980 till 2013.

Aim. To improve the results of treatment of children with bilateral nephroblastoma

Materials and methods. During the period from 1980 to 2013 at the Institute of Oncology and Hematology were examined and treated 82 children with bilateral nephroblastoma.

In all patients, the diagnosis was confi rmed morphologically. The main peak of incidence of bilateral nephroblastoma accounts for the period from age 3 to 5 years – 47 (57.3 %).

All children received neoadjuvant chemotherapy vincristine, dactinomycin, doxorubicin. Surgical treatment was 78 of 82 children. The other four children have not received surgical

treatment due to progression of the disease on the background of the treatment. Prior to surgery all children performed radioisotope study of kidneys and urine test for endogenous

creatinine clearance. 59 children (75.6 %), surgical treatment was carried out in two stages, fi rst at the least aff ected kidney tumor, then – on the contralateral organ. 19 patients

(23.9 %), surgery was performed in one step. Postoperative chemotherapy and radiotherapy (if necessary) is carried out, depending on the stage of disease and histological variants

f the tumor, according to the accepted protocol in the clinic. Since 2010, children with bilateral nephroblastoma, receive treatment protocol International Society of Pediatric Oncology

(SIOP) 2001.

Results. 7 patients were re-operated because of local recurrence. Four of them were anti-relapse chemotherapy after repeated operation and are in dynamic observation over 2 years,

3 patients died of disease progression. 2-year OS rate – 84.7 %, 5-year OS rate – 77 %, 2-year EFS – 79 %.

Conclusion. The correct diagnostic and modern strategy therapy can improve overall survival in children with bilateral nephroblastoma.

A B S T R A C T N O . : O P - 0 9 7

Renal cell carcinoma in children: report of two cases

N.G. Uskova, N.N. Merkulov, S.R. Talypov, D.Yu. Kachanov, A.M. Mitrofanova,

S.R. Varfolomeeva, T.V. Shamanskaya, N.S. Grachev

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: renal tumors, renal cell carcinoma, children

Introduction. Renal cell carcinoma is a rare tumor in children. This disease constitutes 0.1–0.3 % of all tumors and 1.8–6.3 % of renal neoplasm in childhood and adolescent younger

than 15 years.

Aim. To show two cases of the successful treatment of such a rare tumor in pediatric population as a renal cell carcinoma.

Materials and methods. From January 2012 to January 2016 77 patients with renal neoplasm were operated on. On histology the majority of the patients had nephroblastoma –

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57 (74.0 %); also we observed mesoblastic nephroma – 6 (7.8 %), rhabdoid tumor – 5 (6.5 %), clear-cell sarcoma – 4 (5.2 %), carcinoma – 2 (2.6 %), nephroblastomatosis –

1 (1.3 %), cystic nephroma – 1 (1.3 %) and neuroepithelial tumor – 1 (1.3 %).

Case 1. A 6 y.o. girl. On admission complaints of an abdominal pain and a painful urination. Abdominal US and CT scan showed a tumor of the lower pole of the right kidney measuring

41 × 37 × 49 mm (the size of the right kidney was 84 × 30 mm). Chest CT scan showed no metastasis. No pre-existing condition and no inherited syndrome were found. A 4 weeks

of neoadjuvant chemotherapy according to SIOP 2001 protocol for localized stage I of a disease (ACT-D, VCR) were administered. CT scan after chemo showed the same size

and structure of a tumor. The patient was operated on. The surgical procedure included laparotomy, modifi ed radical nephrectomy with preservation of the adrenal gland, biopsy

of lymph nodes of the ipsilateral and contralateral renal hilum and paracaval lymph nodes. Histology was associated with translocation Xp11.2/transformation of gene TFE3

(TFE3 antibody positive reaction on immunohistochemistry, no cytogenetic examination was performed), grade 3, stage I. No postoperative treatment was performed. At the present

time the patient is under observation.

Case 2. A 10 y.o. boy. On admission complaints of a high blood pressure controlled by p.o. intake of enalapril. Abdominal US and CT scan showed a tumor of the upper pole of the right

kidney measuring 38 × 35 × 37 mm (the size of the right kidney was 89 × 36 mm). Chest CT scan showed not numerous subpleural 2–3 mm foci (metastasis?). Bone scintigraphy

showed no metastasis. No pre-existing condition and no inherited syndrome were found. A 6 weeks of neoadjuvant chemotherapy according to SIOP 2001 protocol for unilateral

lesion and distant metastasis (ACT-D, VCR, DOX) were administered. CT scan after chemo showed the same size and structure of a tumor and of lung foci. The patient was operated on.

The surgical procedure included laparoscopy, modifi ed radical nephrectomy with preservation of the adrenal gland, biopsy of lymph nodes of the ipsilateral renal hilum and aortocaval

lymph nodes. On histology it was clear-cell renal cell carcinoma, grade 1, stage I. No postoperative treatment was performed. The lung foci were considered as not metastatic.

At the present time the patient is under observation.

Results. There was no blood loss during both surgery procedures. The duration of the fi rst operation was 90 minutes and of the second one 220 minutes. No intra- or postoperative

complications were observed. In both cases the tumor was less than 5 cm. Both patients were classifi ed as stage I. The post-surgery follow-up is 2 months in case 1 and 1.5 months

in case 2. There is no evidence of relapse according to the 1st US investigation data.

Conclusion. Patients with localized renal cell carcinoma could be treated with surgery alone. But they should receive careful post-operative monitoring of lungs and liver to prevent

a spread of a disease.

A B S T R A C T N O . : P P - 1 2 0

Diagnosis of renal tumours in neonates

I. Begun, I. Papkevich, R. Tarasevich

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: renal tumours, neonates, ultrasound diagnosis

Introduction. Renal tumors are quite common in childhood, ranking fi fth among all pediatric tumors. Wilms’ tumor is the most frequent renal tumor in childhood (represents 95 %

of kidney tumors). Wilms’ tumor is the more frequent in infants till 1 year and are its ratio with mesoblastic nephroma is about 2:1. But if we focus on the age group of infants under

6 months this ratio already can be is 1:2.

Aim. The aim of this study to learn the frequency and sonographic characteristics of main types of human embryonic tumors of kidney that manifest themselves in the perinatal and

neonatal periods.

Materials and methods. A retrospective analysis data of fi rst month of life infants with a confi rmed diagnosis of a solid tumor of kidney was made. Depth of retrospective

was 15 years.

Results. Proportion of patients with major forms of kidney tumors in the neonatal period amounted to 3.4 % from all children till 14 years with this disorder. These were 7 cases with

a confi rmed diagnosis of a solid tumor of kidney in neonates. In 6 of 7 of cases in neonates with renal tumors had mesoblastic nephroma. All 6 cases clinically have been classifi ed

as “defi nitely congenital” tumors, 3 of which were diagnosed prenatally at 33–35 weeks of pregnancy. On postnatal sonograms were determined the rounded forms solid tumors

of the kidney 9–40 cm3. Acoustic tumor density was middle or low, degree of vascularization was average or high. Indices of resistance for arteries in diff erent sections of tumors

were ranged from 0.50 to 0.79. If tumor was localized in the upper segment of the kidney we need to diff erentiated it and the neuroblastoma adrenal. We observed only one case

of neonatal nephroblastoma. It was coming from lower pole of the left kidney, a solid, with a fuzzy on the border with parenchyma of kidney contour of the tumor. Tumor volume was

15 cm3. Were determined of average tumor vascularization, deformation and expansion of renal pelvis, lymph nodes along renal pedicle. Was marked by rapid growth of neonatal

renal tumor (tumor volume doubling time was 8–13 days). At this point it should be noted that in infants till 1 year in the ¾ cases tumors of kidney they have been diagnosed

in volume exceeding 250 cm3.

Conclusion. Ultrasound as the primary method of postnatal (neonatal) diagnosis of kidney tumors in all cases was. Prenatal detection of the visually similar (on malignant) benign

forms of the renal neoplasms is indicate on a certain effi ciency of the prenatal diagnosis of the renal tumor as such. 50 % eff ectiveness of prenatal ultrasound diagnosis for neonates

with mesoblastic nephroma were noted. There is a low frequency of occurrence of nephroblastoma in the newborn (0.48 % of all cases of this tumor). Mesoblastic nephroma

predominates in a ratio of 6:1. Signifi cant dimensions of tumors in infants till 1 year with one hand and a low incidence of neonatal nephroblastoma with other hand are confi rming

the high growth rate of the nephroblastoma in age group till 1 year.

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A B S T R A C T N O . : P - 2 5 5

The fi rst experience of nephron-sparing surgery in children

with Wilms tumor using a single laparoscopic access

N.R. Akramov, A.K. Zakirov, R.Z. Shammasov, E.I. Nizamutdinova

Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan

Key words: Wilms tumor, partial nephrectomy, nephron-sparing surgery, children

Introduction. In recent years, the question of preserving treatment in children with Wilms tumor is being discussed in the international literature. Unfortunately, preserving surgery

can be performed not in all patients. But the surgical techniques improve at an incredible rate. Of course, the problem of nephron-sparing surgery in children with Wilms tumor

is not limited by the improvement of surgical techniques. Oncohematologists, chemo- and radiotherapists, anesthetist, pathologists and other specialists are working on the problem.

Only the joint eff orts of various experts can cure the patients with such disease.

Aim. To identify the use of nephron-sparing single laparoscopic access surgery of children with Wilms tumor.

Materials and methods. Two children (3 and 4 years-old) with Wilms tumor were treated in the Children’s Republican Clinical Hospital of Tatarstan Republic in 2015.

Pre- and postoperative therapy was carried out in accordance with the Protocol SIOP 2001 / GPOH. The decision about the preserving surgery in the fi rst case was made due

to the bilateral process and in the second case to the volume of tumor lesions less than 30 % of the kidney. In both cases, as a surgical technique were performed partial nephrectomy

by a single laparoscopic access. In both cases, the tumor was located in the left kidney. That is why the access to the abdominal cavity was performed through a 2.5 cm length incision

on the left semicircle of umbilicus, using SILS-port (Covidien, USA), and CO2 insuffl ation into the peritoneal cavity up to 10 mm Hg, installation of the 5 mm laparoscope 30°. After the

abdomen revision, the left retroperitoneal space was opened from splenic angle on the lateral border of the colon using dissector-roticulator, clinch-roticulator and ultrasonic scissors

Harmonic (Johnson & Johnson, USA). Doing so, the upper pole of the left kidney was visualized. Initially, the ultrasonic scissors and dissector denuded the renal pedicle. The rubber

band was applied on the renal vessels with the exposition of no more than 40 minutes. All the tumor arteries and veins extending from the renal pedicle were clipped and intersected.

The tumor was dissected entirely after the renal resection at the level of the healthy parenchyma (5 mm from the tumor). The tumor was removed using a laparoscopic evacuation bag

(in one case skin incision was increased to 4 cm). A retroperitoneal sanation was carried out. The renal wound was closed with hemostatic mesh and was sprinkled with hemostatic

powder. The top of the wound were applied with kidney fat. Then the rubber band was removed from the renal pedicle. Intraoperative blood loss in both cases were about 25 ml.

Results. The removed renal segment with a tumor in the fi rst case had a size of approximately 45 × 30 mm (weight about 50 g), in the second case about 95 × 80 mm (weight about 220 g).

Six months later the surgery both children were tested on the computerized tomography, which showed no signs of metastasis or recurrence of the tumor. Parents of the patients were

satisfi ed by the cosmetic results of the surgical treatment.

Conclusion. Thus, the partial nephrectomy in children with Wilms tumor using of a single laparoscopic approach may be the choice with good functional and cosmetic results.

A B S T R A C T N O . : P P - 2 6 0

Renal tumors in infants less than 6 months of age

Merazi Nassima, Lamia Sabrina Khesrani, Yasmina Ladjadj

Hospital Mustapha Pacha, Alger, Algeria

Key words: nephroblastoma, mesoblastic nephroma, infants

Introduction. Renal tumors are rare in infants less than 6 months of age. Nephroblastoma and mesoblastic nephroma (MN) are the most common tumors.

Aim. The aim of this study is to determine their frequency in this age group, and to defi ne their clinical, histopathological characteristics, treatment of these tumors and outcomes

of the patients.

Materials and methods. In this retrospective study, performed in pediatric surgery department of university hospital Mustapha Pacha of Algiers, we reviewed our 20 years

experience, from january 1996 to december 2015.

Results. Among 23 cases of renal tumors in this category of patients, the nephroblastoma leads in the fi rst position (15 cases), and 8 patients suff ered from mesoblastic nephroma.

the mean age was estimated at 3.24 months. The female prevalence was noted with a sex ratio of 0.7 (13 females against 10 males). In all these cases, the main symptom consisted

of abdominal mass. All the 23 patients underwent a widened nephro ureterectomy; 14 of them had fi rst surgery (8 MN and 6 nephroblastoma). The 9 other cases underwent

preoperative chemotherapy. The most common of histologic type found was intermediate (6 cases). However 5 patients presented nephroblastoma with unfavorable histology.

All patients with mesoblastic nephroma are alive, and 3 with nephroblastoma died; one of them following a head trauma.

Conclusion. In our study, the renal tumors observed in infants less than 6 months of age represente 6.33 % of all tumors of kidney in the child. We have noted a high frequency

of nephroblastoma (65.2 %) compared to the mesoblastic nephroma which takes the second rank in this type of tumors. This study has been carried in order to improve the medical

care of the patients. In this age group, we have to think fi rst of malignant tumors, and to better analyze the imaging supports and the extension of the tumors.

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A B S T R A C T N O . : O P - 2 6 6

Neoadjuvant transcatheter arterial chemoembolization combined with systemic

chemotherapy for treatment of clear cell sarcoma of the kidney (CCSK)

Minju Li, Jinhu Wang, Daxing Tang, Shan Xu, Junqing Mao, Can Lai, Qiang Shu

Children’s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China

Key words: transcatheter arterial chemoembolization, clear cell sarcoma of the kidney

Introduction. Clear cell sarcoma of the kidney (CCSK) is a rare type of pediatric malignant renal tumor. It is known as an aggressive tumor with poor prognosis.

Aim. The aim of the study was to evaluate the effi cacy of neoadjuvant transcatheter arterial chemoembolization (TACE) combined with systematic chemotherapy for the treatment

of CCSK in children.

Materials and methods. From January 2010 to December 2015, 5 patients (2 boys and 3 girls; median – 2.9 years, range 0.9–7.1 years) with unilateral CCSK were treated with

preoperative TACE combined with systemic chemotherapy. All of these patients were diagnosed histologically by percutaneous core-needle biopsy before treatment. At diagnosis,

the mean maximal tumor diameter was 11.7 cm (range: 6.7–14 cm). Two patients (2/5) presented with lung metastasis, 1 (1/5) with bone metastasis, and 1 (1/5) with inferior vena

cava (IVC) thrombus.

Patients subjected to TACE by Seldinger’s method. A catheter was placed into the involved renal artery. Chemoembolization emulsion consisted of cisplatin (80–90 mg/m2), pirarubicin

(40 mg/m2), vindesine (3 mg/m2) , normal saline (120–180 ml) and iodized oil (5–10 ml) were infused into the renal artery. Preoperative systemic chemotherapy with vindesine

(3mg/m2/day, on day 1), ifosfamide (1200mg/m2/day, on days 2–4), and etoposide (100mg/m2/day, on days 5–7) was administered 3 weeks after TACE. Surgical resection carried out

3 weeks after neoadjuvant intravenous chemotherapy. Postoperative received radiotherapy and chemotherapy with a combination of carboplatin, ifosfamide, etoposide, pirarubicin,

vindesine for 24 weeks.

Results. No cardiotoxicity, renal insuffi ciency, or hepatic dysfunction were found in all patients. Grade III marrow suppression developed in 2 patients. In terms of RECIST criteria,

3 patients had a partial response (PR), 1 had stable disease (SD) and 1 showed progressive disease (PD) after neoadjuvant therapy. Four patients underwent radical nephrectomy

after neoadjuvant therapy. Complete surgical removal of the tumor achieved in 3 patients and 1 had intraoperative tumor rupture. Surgical stages of this 4 patients were stage II in

1, stage III in 2, and stage IV in 1 patients. Pathologic examination of surgical specimens found tumor necrosis > 90 % in all 4 patients. This 4 patients were recurrence free survival

up to now with a median follow-up of 44 months (range, 25–62 months). Another patient with tumor progress after neoadjuvant therapy failed to surgery, died of lung metastasis

and bloody ascites.

Conclusion. Neoadjuvant transcatheter arterial chemoembolization combined with systematic chemotherapy for the treatment of clear cell sarcoma of the kidney in children is safe

and eff ective.

A B S T R A C T N O . : O P - 2 6 8

Inhibition of autophagy in nephroblastoma and the potential therapeutic signifi cance

Xi Chen, Jinhu Wang, Linjie Li, Min Yang, Duo Zhou, Yilong Wang, Qiang Shu

Children’s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China

Key words: nephroblastoma, pediatric tumor, autophagy, chloroquine

Introduction. Nephroblastoma (NB) is the most common abdominal malignant neoplasms and contributes to 6 % of malignant pediatric tumors. Currently, it is treated by

surgery in combination with chemo- and radio-therapy. NB has overall good prognosis, however, patients still suff er from the side eff ects of traditional therapies. Autophagy

is a self-digestion physiological event to maintain cellular homeostasis. Deregulation of autophagy has been proved to participate in the onset and progression of various malignant

tumors. Investigations of the autophagic activity in NB have not been reported in the literature. However, abnormal expressions of Bcl-2, β-catenin and p53, which directly regulate

the autophagy activity, are manifested in NB. Hence we hypothesize a potential link between autophagy deregulation and the onset of NB. Furthermore, regulation of autophagy can

be a potential strategy as adjuvant therapy to increase the effi cacy and to decrease the side eff ects of traditional therapeutics.

Aim. Our study attempts to explore the relationship between autophagy and NB with the purpose to fi nd optimized protocol by autophagy regulation.

Materials and methods. The following experiments were performed.1. Expression of several autophagy-related genes (ATGs) were analyzed in the mRNA and the protein levels

in NB tissues. 2. In NB cell lines, novel therapeutic strategies were tested by combinational use of autophagy-targeting drugs and conventional chemotherapeutics to increase

the effi cacy and to decrease the side eff ects. 3. In nude mice NB tumor model, the synergistic antitumor eff ect of chloroquine, a typical autophagy-suppressant, with the antimetabolite

vindesine was tested.

Results. 1. There was suppression of autophagy in NB tissues prior to chemotherapy compared with the surrounding normal kidney tissues; autophagy was activated after

chemotherapy and might participate in chemoresistance development. 2. The microtubule-targeting antimetabolites vincrestine and vindesine had synergistic antitumor eff ect with

both chloroquine (autophagy-suppressing drug) and rapamycin (autophagy-promoting drug). The anti-proliferative eff ect of rapamycin on NB was at least partly through activation

of autophagy. 3. The synergistic antitumor eff ect of chloroquine with vindesine was confi rmed by in vivo experiments in nude mice.

Conclusion. Through this project, a standardized tumor tissue bank has been built in our hospital and will serve as the tool and the resource for future research. We have examined

the background autophagy levels in the NB tissues, and found autophagy was suppressed and could be re-activated by chemotherapy. In both in vitro and in vivo experiments,

inhibition of autophagy (by chloroquine) was shown to decrease tumor growth synergistically with microtubule-targeting chemotherapeutics. From this project, new insights were

gained in the tumorigenesis and chemoresistance mechanisms of NB, and novel therapeutic strategies could be further investigated based on the current data.

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A B S T R A C T N O . : O P - 2 9 5

Rare pediatric renal tumors: always keep in mind. An experience of one laboratory

A.M. Mitrofanova, D.M. Konovalov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: renal tumors, rare tumors, pediatric renal tumors

Introduction. The most well known and diagnosed pediatric renal tumor is nephroblastoma. But wide age limits in childhood (0–18 years) should always be taken into account

to not miss a rare tumor.

Aim. To show wide spectrum of pediatric renal tumors of all ages in group, including newborns and older children.

Materials and methods. 231 renal tumors were studied in our department since January 2012. There were 187 pediatric cases including 77 children operated on in our clinic.

Patients’s age varied from 1 month to 17 years. Only 100 entire kidneys were delivered to the laboratory and could be examined fully and carefully. The rest of tumors were biopsied or

cut not in the right way so they couldn’t be graded. Histologically there were nephroblastoma (119 – 63.6 %), rhabdoid tumor (14 – 7.5 %), nephrogenic rests (14 – 7.5 %), congenital

mesoblastic nephroma (11 – 5.9 %), clear cell sarcoma of kidney (10 – 5.3 %), translocational carcinoma (5 – 2.7 %), metanephric adenoma (5 – 2.7 %), clear cell carcinoma

(2 – 1.06 %), cystic nephroma (2 – 1.06 %), high grade sarcoma NOS (2 – 1.06 %), medullary carcinoma (1 – 0.5 %), juxtaglomerular cell tumor (1 – 0.5 %), metanephric stromal

tumor (1 – 0.5 %). Almost in all consultative cases there was no clinical information except age and location of the tumor. All the tumors were diagnosed histologically and in some

cases by using immunohistochemistry as well.

Results. The diagnosis was confi rmed in 107 cases (57.2 %), 52 tumors were diagnosed primary (27.8 %), in 28 cases the incoming diagnosis was changed cardinally (14.9 %).

None of benign tumors were diagnosed correctly by the local pathologist or suspected by clinician. None of consultative rhabdoid tumors were diagnosed in local departments

of pathology and only half of it were suspected by clinician.

Conclusion. Not only nephroblastoma should the fi rst diagnosis in pediatric renal tumors. The patient should be examined fully and, moreover, complete clinical information must

be provided to the pathologist to avoid rough diagnostic mistakes and misdiagnosing the tumor.

A B S T R A C T N O . : O P - 3 3 0

Five year experience of Wilms tumor at a tertiary care centre, where we stand?

A developing country perspective

Saadia Anwar1, Mahwish Faizan1, Safi a Khan1, Agha Shabbir Ali1, Ahsan Waheed Rathore1, Alia Ahmad1, Mary Taj2

1The Childrens Hospital and ICH, Lahore, Pakistan; 2The Royal Marsden Hospital, Sutton, England

Key words: WT

Introduction. We present a retrospective study, looking at demographics and outcome of children with WT presented to the hematology and oncology department of the Children’s

Hospital Lahore between January 2009 and December 2013.

Aim. The main objective of the study is to discuss presentation and outcome of children with Wilms tumor (WT).

Materials and methods. All children diagnosed as WT on radiological fi ndings and proven on histopathology after needle biopsy were included. Data regarding age, gender, initial

staging and outcome were recorded and analyzed. CT scan abdomen, chest and needle biopsy were done in almost all patients. According to SIOP WT 2001 (UKCCSG) Protocol all

patients were treated with Pre-operative chemotherapy, surgery followed by post-operative chemotherapy and radiotherapy in some cases.

Results. A total of 175 patients were included. Males and females were equal in number 51.4 % and 48.6 % respectively. Majority 88 (50 %) were between 2–5 years of age,

44 (25 %) patients were above 5 years and 43 (24.6 %) below 2 years. Sixty two (36 %) presented in stage III, 37 (21 %) stage IV metastatic disease, 26 (15 %) stage II and only

5 % in stage I and V each. Seventy four (42.2 %) patients had completed treatment advice, 20 (11.4 %) died and 10 (5 %) had relapse while 65 (22.2 %) were lost to follow up with

missing record.

Conclusion. Most of patients presented at less than fi ve year of age with advance disease stage III and IV. Treatment outcome is fair 42.2 % with abandonment and 67 % without

abandonment with overall mortality 11.4 %. Abandonment due to multiple social reasons is major factor aff ecting the overall survival rate that need to be addressed to improve

ultimate outcome.

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A B S T R A C T N O . : O P - 3 5 5

The treatment of Wilms’ tumour, a single institution experience

L.I. Shats, M.B. Belogurova

City Clinical Hospital № 31 of Saint-Petersburg, Russia; Saint-Petersburg State Pediatric Medical University, Russia

Key words: paediatric renal tumours, Wilms’ tumour, nephroblastoma

Introduction. There is no national protocol for treatment of Wilms’ tumour (WT) in Russian Federation yet. The Department of Paediatric Haematology and Oncology of Clinical City

Hospital 31 is not a participant of SIOP 93-01/GPOH and SIOP WT 2001 but we were allowed to follow diagnostic and treatment recommendations of these protocols.

Aim. The aim of our study was to evaluate the results of a treatment in patients with WT who were treated according to the protocols in City Hospital 31 in St.Petersburg.

Materials and methods. We analyzed a retrospective data review of 51 patients (pts) with WT over a period of 16 years (1999–2015). There were 28 girls and 23 boys in our group.

Forty seven pts developed an unilateral WT and in 4 pts both kidneys were involved. The median age was 2 years (1 mo – 8 yrs). A pre-treatment aspiration biopsy was provided

in 23 children (45 % cases).

Results. The median follow up time was 42 months (1–178 months). Five years event free survival (EFS) was 84.9 ± 5.2 % and the overall survival was 86.9 ± 5.7 %.

The EFS of children with stage I (5 pts) was 100 % and it was 95.2 ± 4.6 % in stage II tumor group (24 pts). EFS at 5 years for stage III (8 pts) and IV (12 pts) were 87.5 ± 11.7 %

and 58.3 ± 14.2 % respectively. Two patients with bilateral WT without lung metastases are in the complete remission now and have a normal renal function. There were only 2pts

in the low risk group and both are alive. EFS at 5 years for 42 children in the intermediate risk group (IRG) was 91.8 ± 4.5 % and 50.0 ± 20.4 % for 6pts in the high risk group (HRG).

Six pts relapsed. The median time of recurrence was 7 months: one child achieved a second remission (in IRG) but was lost to follow up, 4 pts died of the recurrence (in HRG),

and another patient from IRG with a local relapse of stage II WT continues his treatment now. There were 2 treatment-associated deaths: 1st- during initial preoperative chemotherapy

from disseminated intravascular coagulation, 2nd- in complete remission of bilateral WT stage IV (IRG) due to an acute intestinal obstruction at the end of the postoperative

chemotherapy.

Conclusion. The present study demonstrates the favorable outcome in the local stage cases with an initial preoperative chemotherapy followed by a surgery and a postoperative

chemotherapy. However, in the stage IV cases and in the high risk group the survival rate is not satisfactory.

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A B S T R A C T N O . : P P - 1 0 4

Level of the TFPI-2 mRNA expression and VEGF-A165/VEGF-A189 ratio

in pretreatment tumour tissue is an independent predictor of poor prognosis

for pediatric patients with nonmetastatic Ewing’s sarcoma

L. Kisialeu, T. Savitskaya, N. Lipay, O. Aleinikova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: angiogenesis, pediatric patients, Ewing’s sarcoma, prognostic marcers

Introduction. The improvement of Ewing sarcoma (ES) therapy is currently linked to the selection of patients who are likely to develop disease recurrence and of modifi ed treatment

regimens for them. Vascular endothelial growth factor (VEGF) as well as tissue factor pathway inhibitor (TFPI) are known marker of carcinogenesis. We have previously shown that

TFPI-2 mRNA (messenger ribonucleic acid) expression and VEGF-A165/VEGF-A189 mRNA ratio were signifi cantly diff erent in the metastatic versus nonmetastatic forms for children

with malignant diseases of bones and soft tissues. We hypothesized that these biological markers may have prognostic value exclusively for patients with nonmetastatic ES.

Aim. In this study we analyzed the ability to predict the response of ES to systemic therapy based on molecular markers.

Materials and methods. The mRNA expression level of TFPI-2 and ratio of VEGF-A165/VEGF-A189 isoforms in pretreatment tumour tissues from 20 nonmetastatic ES pediatric

patients were studied by quantitative reverse transcriptase–polymerase chain reaction. The value P ≤ 0.05 was regarded as statistically signifi cant. Kaplan–Meier and log-rank

methods were used, respectively, to draw and evaluate the signifi cance of survival curves in patients with ES.

Results. It was identifi ed molecular combination that refl ect tumor resistance to conventional drugs before systemic treatment. We observed that low mRNA expression level

TFPI-2 (≤ 0.8) and VEGF-A165/VEGF-A189 ratio (≤ 1) was a signifi cant independent negative prognostic indicator of event-free survival (EFS) rate. For patients with nonmetastatic

ES and molecular combination of poor prognosis 5-year EFS rate was 27 %, while for other patients it was 89 % (P ≤ 0.05).

Conclusion. Discriminating patients with nonmetastatic ES into poor- and standard-risk groups possible to carry before systemic treatment based on angiogenesis markers:

the mRNA expression level of TFPI-2 and ratio of VEGF-A165/ VEGF-A189 isoforms. This will provide an opportunity to intensify treatment for patients with poor prognosis.

BONE TUMOURS

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A B S T R A C T N O . : P - 1 3 4

Endoprosthetic reconstruction of knee joint in children and adolescents with primary

bone tumors in Federal Research Center of Pediatric Hematology, Oncology and

Immunology named after Dmitriy Rogachev

A.I. Karachunskiy, N.A. Bolshakov, N.S. Grachev, M.V. Tikhonova, K.S. Tsilenko

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: endoprosthetic reconstruction, primary bone tumors, knee joint, children, adolescents

Introduction. Endoprosthetic reconstruction is the most common method of limb-sparing surgery in oncology, including practical pediatrics. Primary bone tumors localized

in metaphyseal areas require joint arthroplasty. Bone tumors in patients with sceletal immaturity occur in the metaphyseal region, close to the growth plate, so that sacrifi ce of a major

plate often is necessary when tumor is excised. These patients need expandable prostheses.

Aim. Show own experience of endoprosthetic reconstruction of knee joint in children and adolescents with primary bone tumors.

Materials and methods. Since June 2012 in our surgery department 38 endoprosthetic reconstructions of knee joints have been performed. Among them 27 endoprosthetic reconstructions

of the distal femur, including 10 cases with the use of “growing” non-invasive endoprosthesis; 11 endoprosthetic reconstructions of proximal tibia, including 5 cases using “growing” non-

invasive endoprosthesis. The youngest patient was 7 years old, the oldest – 17 years old. The MSTS scale was used for evaluation of functional results in 3 months after operation.

Results. During endoprosthetic reconstruction in the case of distal femur sarcomas in 25 patients range of volume replacement ranged from 160 to 315 mm. proximal. Range

of proximal tibia replacement was 140–160 mm. In all cases, the results of histological examination of the resection margins showed no tumor cells, but 4 patients were diagnosed

with progressive disease. 1 patient after 12 months was diagnosed with local relapse and required rotation plasty. All patients have started rehabilitation from the fi rst days after

surgery. The worst functional outcome scale MSTS after 3 months was – 50 %, the best – 93 %. Average – 76 %. At present time, just 6 patients with “growing” endoprosthesis have

require extension, which was performed without any problems.

Conclusion. Limb-sparing surgery in children with oncological diseases of the musculoskeletal system is the preferred method of treatment. Endoprosthesis secures good oncological

and functional results, as well as favors the most adequate social adaptation of a child. The use of modern oncological systems for arthroplasty in pediatric and adolescent surgical

practice may achieve good oncological and functional results.

A B S T R A C T N O . : P P - 3 0 4

The role of radiation therapy in treatment of pediatric and young adult patients

with Ewing sarcoma family tumors

T. Jukhta1, Yu. Punanov1, I. Kazantsev1, A. Malinin2, S. Safonova1, G. Gafton2, A. Gevorgyan1,

E. Morozova1, L. Zubarovskaya1, B. Afanasyev1

1Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia; 2N.N. Petrov Research Institute of Oncology, St-Petersburg, Russia

Key words: Ewing sarcoma, radiation therapy

Introduction. Ewing sarcoma family tumors (ESFT) have aggressive clinical course and tend to disseminate early, which is the reason for many advanced or metastatic

primary cases (about one third of patients). ESFT treatment programs combine dose-intensive systemic therapy with aggressive local control possibly sparing the limb function

(surgery and/or irradiation).

Aim. In this study, we evaluated radiation therapy eff ectiveness based on total dose (TD) and time-dose-fractionation (TDF) values.

Materials and methods. A total of 115 children (a median age of 10.5, range 2–17 years) with ESFT received treatment during the period of April 1985 – August 2013.

These patients are divided into two groups based on therapy intensity. The 1st group patients (n = 64) received therapy according to modi-fi ed Т9 protocol, to 2nd group belong

51 patients receiving treatment according to EICESS-92 and Euro-EWING 99 protocols. Sixty-four (56 %) patients underwent primary lesion irradia-tion, 27 (23 %) surgical resection

and 21 (18 %) patients received a combination of both. The target TD was achieved by megavoltage external beam therapy in 5–6 weeks (by daily fractions of 1.8–2.0 Gy given 5 days

a week). A median TDF value on the whole bone and soft tissue component was similar in the 1st (82.3, range 52–103 Gy) and the 2nd (825, range 59–108 Gy) group. In patients with

axial tumor the doze was conducted to the whole bone. If the lesion was limited to metaphysis or metadiaphysis, the non-involved epymetaphyses with epiphysial plate was excluded

after reaching TD of 30 Gy to longitudinal axis with boost to the lesion up to 50–55 Gy. All patients with metastatic disease (n = 11) received radiation therapy to all visible lesions.

Results. The most important factors for long-term survival were disease stage, therapy regimen used and disease recurrence (local or systemic). Although there was no diff erence

in event-free survival (EFS) between the groups (37.9 % and 39.5 %, P = 0.25), the overall survival (OS) was better in the 2nd group, than in the 1st one (55 % and 25 % accordingly,

P = 0.03) due to better results in primarily disseminated disease patients. While there was no signifi cant diff erence localized tumor treatment results (OS 49.5 % and 69.9 %,

P = 0.11; EFS 48.6 % and 48.8 %, P = 0.6 for 1st and 2nd groups patients accordingly), all patients with metastases from the 1st group died 45 months after the end of therapy, while

in the 2nd group OS and DFS are 45.8 % and 28.9 %, accordingly. Local and systemic relapse rate in localized disease patients was similar for both groups (30 % and 25 %, 52 %

and 58 %, accordingly), better result were generally observed in patients with most intensive local control combining surgery and irradiation (18.2% of local relapses).

The local control rate in patients receiving only irradiation was also evaluated based on TD and TDF values. Local relapse cumulative incidence in patients with TD values of

Conclusion. Unlike TD, TDF values showed direct correlation to local relapse rate with a minimal of 12.6 % reached in > 80 TDF group. This method of biologically equivalent dose

calculation may prove valuable for local control planning in ESFT patients.

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A B S T R A C T N O . : P P - 3 3 1

Preliminary treatment results of patients with malignant bone in Federal Research Center

of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev

M.V. Tikhonova, D.V. Rogozhin, A.S. Slinin, N.V. Zhukov, D.V. Litvinov, N.A. Bolshakov, K.S. Tsilenko,

M.V. Teleshova, A.N. Remizov, A.V. Nechesnyuk, A.I. Karachunskiy

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: osteosarcoma, Ewing’s sarcoma

Introduction. In Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev a scientifi c clinical group for bone tumors is formed and

works. The infrastructure of the Centre allows to perform a full complex of examinations, treatment and rehabilitation of patients with malignant locomotor system tumors including

all types of imaging, molecular genetic studies, diff erent types of chemotherapy, targeted, high dose and radiation therapy, as well as all current methods of surgical treatment.

A therapy study of patients with bone tumors within EURO EWING 2008 and EURAMOS-1 protocols begun from September 2012.

Aim. To evaluate a programmed therapy effi ciency in patients with osteosacoma and Ewing’s sarcoma.

Materials and methods. From September 2012 79 patients with bone tumors were included in the study (Ewing’s sarcoma – 30, osteosarcoma – 49) aged from 5 to 19. Exclusionary

criteria: previous treatment according to other therapy regimens, timing breach, age of patients under 5 years, treamtent refusal of patients or their parents, osteosarcoma

and Ewing’s sarcoma of non-born localization. From the patients with osteosarcoma enrolled to the study, 41 (84 %) patients had conventional osteoblastic osteosarcoma, conventional

chondroblstic – 4, fi broblatic – 1, teleangiectasic – 2, parosteal – 1 among osteosarcoma patients enrolled in the study. All histological diagnoses were verifi cated in Federal Research

Center of Pediatric Hematology, Oncology and Immunology as a reference centre. Stage distribution: I stage – 1 , IIА – 9, IVВ – 13, III – 3, IVA – 14, IVB – 8. Histologically 28 patients

have Ewing’s sarcoma among patients with Ewing’s sarcoma. Primitive neuroectodermal tumor is in 2 patients. Accordign to stage: I – 2 patients, IIA – 7, IIB – 8, III – 1, IVA – 4, IVB – 8.

Patients received a therapy within EURAMOS, EURO EWING 2008 protocols, respectively.

Results. At the moment 3-year event-free survival is 55 % in the osteosarcoma group and 25 % – in the Ewing’s sarcoma group. All deaths resulted from main disease progression.

No patient died as a result of acute treatment toxicity, severe late eff ects were not reported.

Conclusion. Current methods and approaches to malignant bone tumor therapy in children are available in Federal Research Center of Pediatric Hematology, Oncology

and Immunology named after Dmitriy Rogachev, that allows the Centre to participate in international studies.

A B S T R A C T N O . : O P - 4 0 3

Experience in the treatment of Ewing’s sarcoma of the pelvis in children.

East-European Sarcoma Group

D. Nisichenko, A. Dzampaev, O. Nisichenko, V. Khayrullova, D. Hestanov, M. Aliev

N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: Ewing’ sarcoma, pelvis, VAC, IE

Introduction. Ewing sarcoma of the pelvis is one of the worst locations for treatment in children

Aim. The aim of our study was to determine treatment outcomes in two protocols: 1) using high-dose chemotherapy and autologous peripheral blood stem cell transplantation

(HDCT/autoPBSCT) and 2) protocol with decreasing doses of chemotherapy in pediatric patients with Ewing’s sarcoma of pelvis treated at the East-European Sarcoma Group

(N.N. Blokhin Russian Cancer Research Centre).

Materials and methods. We retrospectively analyzed the data of patients with Ewing’s sarcoma of pelvis between 1997–2011. All patients receive the same mode neoadjuvant

chemotherapy regimens 5 courses (VAC and IE).

Results. The fi rst group included 31 children. Metastatic disease was found in 42 % of patients. The average tumor volume was 345 ml. Middle age was 11.5 y.o.

The second group included 21 patients with a reduction in drug dosage. Metastatic disease was found in 33 % of patients. The average tumor volume was 298 ml. Middle age was 12.7 y.o.

Overall 1-, 3-, 5-y survival after HDCT/autoPBSCT were 70 %, 39 %, 39 %. Median was 17.2 months in fi rst group. With metastatic disease overall 1-, 3-, 5-y survival were 46 %, 7 %,

7 %. Median was 10.2 months. With non metastatic disease overall 1-, 3-, 5-y survival were 88 %, 64 %, 64 %. Median not reached.

Overall 1-, 3-, 5-y survival in the second group were 100 %, 70.5 %, 58.8 %. With metastatic disease overall 1-, 3-, 5-y survival were 100 %, 68.5 %, 34.2 %. Median not reached.

With non metastatic disease overall 1-, 3-, 5-y survival were 100 %, 71.4 %, 71.4 %. Median not reached. Data is valid (P = 0.008).

Conclusion. Although we obtained the best survival data in the second group than the fi rst using HDCT/autoPBSCT, are diff erent by selection the data volume of the primary tumor

and rate of metastatic disease. Requires further study and monitoring of patients undergoing treatment for Ewing’s sarcoma of the pelvis.

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A B S T R A C T N O . : P P - 0 9 4

Experience of high-dose chemotherapy and autologous hematopoietic stem cell

transplantation of pediatric patients with high risk rhabdomyosarcoma

Seung Min Hahn, Won Kee Ahn, Ju Yeon Lim, Jung Woo Han, Moon Kyu Kim, Chuhl Joo Lyu

Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea

Key words: rhabdomyosarcoma, autologous transplantation

Introduction. The treatment of rhabdomyosarcoma is guided on the ‘risk group’ assessment. Intermediate risk group patients with embryonal rhabdomyosarcoma have shown

more than 80 % of survival rates while alveolar less than 60 %. Parameningeal rhabdomyosarcoma is also known as poor risk factor. High risk patients with metastases have

poorer prognosis (lower than 50 % of survival) with current chemotherapy and local control methods. Hence several new approaches are required for the treatment of intermediate

to high risk group rhabdomyosarcoma. Although several studies have failed to improve the outcome with high dose chemotherapy (HDCTx) and autologous hematopoietic stem cell

transplantation (HSCT) in rhabdomyosarcoma, there are controversial opinions of the treatment.

Aim. To fi nd the possible role of HDCTx, this study reviewed the experience of HDCTx followed by autologous HSCT in pediatric patients of newly diagnosed, high risk rhabdomyosarcoma.

Materials and methods. We reviewed 20 cases of high risk rhabdomyosarcoma patients treated with HDCTx followed by autologous HSCT between 2002 and 2014 in our hospital.

All stage IV patients and intermediate group with parameningeal tumors or alveolar pathologic subtypes were classifi ed as high risk group in the study. The primary end point was

overall survival (OS), which was defi ned as the duration of diagnosis to time of death by any cause. The secondary end point was event free survival (EFS). EFS was defi ned as the time

from diagnosis to disease progression, recurrence or death. Data were analyzed using the Kaplan–Meier method, and tested by log-rank test.

Results. Median age of the patients at diagnosis was 6.13 years old (range, 0.56 to 15.1 years), median age at transplantation was 6.85 years old (range, 1.21 to 15.6 years).

Fifteen patients were stage IV, 3 patients were stage III with alveolar subtype, 1 patient was stage I with alveolar subtype and 1 patient was stage III with parameningeal tumor.

Twelve patients received 2 or 3 times of HDCTx following HSCT while 8 patients received one course of HDCTx. Carboplatin, etoposide, with melphalan (CEM) and busulfan with

melphalan (BM) chemotherapy were commonly used conditioning methods. Ten patients expired; seven patients died due to disease progression, 2 with unknown cause, and 1 was

transplantation related mortality. OS was 49.5 ± 11.3 %, and EFS was 40.0 ± 11.0 %. The patients with complete remission at the time of HDCTx and HSCT showed better survival rate

than patients with residual disease at the time of transplantation (80.0 ± 12.6 % vs. 20.0 ± 12.6 %, P = 0.015). Age over 10 years old and lower than 1 years old group had poorer

outcome compared with the patients with age between 1 to 10 years old (30.0 ± 14.5 % vs. 70.0 ± 14.5 %, P = 0.029). Bone and bone marrow involvement are known as poor

risk factor of rhabdomyosarcoma and similar results were shown in our report. Patients with bone and bone marrow involvement showed lower survival rate than patients without

metastasis in the sites (12.5 ± 11.7 % vs. 74.1 ± 12.9 %, P = 0.001).

Conclusion. Survival rate of high risk rhabdomyosarcoma patients with HDCTx with autologous HSCT was comparable with historical reports, and some patients resulted in even

better outcome; patients who have shown remission before HDCTx (80 %), and patients with 1 to 10 years old (70 %). Although our study has limitation of small number of patients,

these fi nding suggest potential benefi t of HDCTx and autologous HSCT in rhabdomyosarcoma.

SOFT TISSUE SARCOMAS

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A B S T R A C T N O . : P - 1 1 6

Results of treatment of soft tissue sarcomas at children during 12-years period

V.B. Makhonin1, R.R. Bayramgulov1, 2, A.A. Gumerov2

1Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: CWS, soft tissue sarcomas

Introduction. Soft tissue sarcomas (STS) at children – morphorogically diff erent group of diseases that is also diff erent on the biological behaviour and treatment approaches.

It takes 5–7 % in the structure of oncological diseases. Diseases is diagnosed usually at III-IV stages, prognosis is negative in this case. The most popular method of treatment is usage

of CWS group protocol.

Aim. Our aim was analysis of children with STS treated with program therapy with CWS-2002P protocol.

Materials and methods. During the period 2004 – 2015 in our center it were diagnosed 60 children with STS (males – 34, females – 26) at the age from 20 days to 17 years old.

The majority of them were teated with the help of CWS-2002 (n = 45) protocol, 10 – received non-protocol treatment, 5 – rejected from the therapy. Among the children with

STS, received treatment with CWS-2002, 22 were diagnosed with rhabdomyosarcoma (13 – embrional, 6 – alveolar), 7 – synovial sarcomas, 6 – PNET, 10 – other histological

types. Tumour localization: body – 15 cases, pelvis and urogenital tract – 11, extremities – 11, head and neck – 8. IRS staging system was used: I stage – 7 patients, II – 6, III – 22,

IV – 10. Only surgical treatment were performed for 5 patients, surgery and radiotherapy – 1 patient. Other patients received chemotherapy with or without radiotherapy. According

the CWS-2002 criterions 1 patient received treatment with low risk (LR), 5 patients – standard risk (SR), 29 – high risk (HR) and 10 – very high risk (VHR).

Results. Among the patients with LR and SR all children are alive, but late relapse was diagnosed at 1 patient with SR, now this patient in second remission. Among patients with HR

the prolonged remission was diagnosed at 17 patients, 1 patient relapsed and treated, 1 patients alive with signs of tumour; 10 children died: 7 – from disease progression, 2 – from

toxicity of treatment and 1 – from intercurrent disease (AIDS). Among the VHR group 5 patients died from progression, 1 – in the process of therapy, 4 – alive in remission. Relapses

were more frequent in HR and VHR groups – 14 patients from 45 with STS, 10 patients in this group died, 4 alive. Overall survival in the protocol group was 28/45 (62.2 %), relapse

free survival – 24/45 (53.3 %), mortality – 17/45 (37.7 %).

Conclusion. Our data suggests that treatment approaches of CWS-2002P protocol can be done in the conditions of regional center. Results of our study is the same with declared.

The following improvement of STS treatment schemes required especially for patients with HR group.

A B S T R A C T N O . : O P - 1 5 5

Rapidly progressive Kaposi’s Sarcoma in an Iraqi boy received valproic acid:

a case report and review of literature

Likaa Fasih Y. Al-Kzayer1, Peter Keizer2, Farah T. Abdulraheem3, Kenji Sano4, Kenichi Koike5

1Shinshu University; 2Dr. G.B. Cross Memorial Hospital, Eastern Health; 3Al-Hamdaniya General Hospital in Mosul; 4Shinshu University Hospital; 5Shinshu University School of Medicine

Key words: Kaposi’s sarcoma (KS), Classic Kaposi Sarcoma (CKS), Valproic Acid (VPA), Human herpesvirus-8 (HHV-8)

Introduction. Kaposi’s sarcoma (KS), a low-grade vascular neoplasm, is associated with human herpes virus (HHV)-8 Infection. Immunosuppression is an important cofactor

in KS process. Classic KS (CKS) is an indolent disease aff ects commonly the Mediterranean elderly men. However, CKS is exceedingly rare in children and when it occurs is much more

disseminated than adults.

Aim. To our knowledge this is the fi rst pediatric CKS case from Iraq. Our patient was showing an unusual aggressive course of the disease while receiving valproic acid (VPA)

of the potential immune-suppressive eff ect.

Materials and methods. Case presentation: A six-year-old Iraqi boy who had cerebral palsy (CP) and epilepsy since he was 9-month-old, had received VPA to control his seizures.

He developed skin discoloration followed by nodules that disseminated proximally from the lower extremities to groin, face, ears and oral cavity, and then he died from severe

respiratory distress after 110 days from the disease evolution.

Results. KS diagnosis was proved by skin biopsy. As the patient was of Arab-Asian ethnicity, and was HIV-seronegative, accordingly, his condition best fi tted the classic form of KS.

Recent studies showed the link of VPA with the reactivation of HHV-8. Accumulated experimental and clinical data elucidated that VPA induces T-cell suppression.

Conclusion. Our report demonstrates a rare rapidly progressing pediatric CKS case and highlights the possible role of the 5-years administered VPA and its challenging eff ect on

cellular immunity based on recent studies. Thus, VPA could have promoted the development of the CKS in our patient. This report also recalls the need of pediatricians to consider CKS

especially when skin lesion appears on the child’s foot even in countries outside the geographical map of the disease.

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A B S T R A C T N O . : P P - 2 3 7

Application of punctional gastrostomy at children with sarcomas of soft tissue

with localization on head and neck

D.Yu. Kachanov, I.V. Zakharov, T.V. Shamanskaya, T.V. Sergeeva, E.S. Vasilyeva, M.V. Teleshova, D.V. Shevtsov,

R.A. Moiseenko, A.V. Nechesnyuk, N.N. Merkulov, S.R. Varfolomeeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: percutaneous endoscopic gastrostomy, soft tissue sarcomas of the head and neck, children

Introduction. A pediatric patients with soft tissue sarcomas (STS) of the head and neck might need treatment strategies that include multimodality treatment with surgery,

radiotherapy, and chemotherapy. A local spread of the tumor process and the intensive therapy including concurrent chemoradiotherapy can be performed a number of complications

associated with malnutrition.

Aim. The present study was performed to evaluate the possibilities use to percutaneous endoscopic gastrostomy (PEG) in pediatric patients with STS of the head and neck. We spent

analysis indications for PEG, evaluate feasibility staging at diff erent stages of treatment, incidence estimate risk of complications.

Materials and methods. The study included 15 patients of 50 pediatric patients with STS of the head and neck at Federal Research Center of Pediatric Hematology, Oncology

and Immunology named after Dmitry Rogachev from 06.2012 and 12.2015 (42 month). All patients was performed the percutaneous puncture endoscopic gastrostomy (PEG) under

sedation in order to provide nutritional rehabilitation.

Results. Median age at the time of PEG was 54 month (range, 4.8–128.4). Twelve patients had rhabdomyosarcoma (80 %), two rhabdoid tumor (13.3 %), and one undiff erentiated

pleomorphic sarcoma (7.7 %).Median time from diagnosis to staging PEG was 18 days (range, 6–143). Specifi c indications for PEG included radiotherapy on the primary tumor site,

malnutrition due to problems oral feeding due to the anatomical location of the tumor. All patients was performed PEG at the stage of intensive care. 1/15 (6.7 %) received relapse

treatment, 1/15 (6.7 %) – radiation therapy. One of 15 patients had pancytopenia (neutrophil levels of less than 1 thousand/ml). He had not complications in the postoperative period.

3/15 (20 %) at the time of PEG received parenteral nutrition. 1/15 (%) – enteral nutritional support through via nasogastric tube. PEG tubes were successfully inserted into all patients.

A feeding by PEG was started two days later. Immediate complications occurred in four (27 %) patients: 2/15 (13.3 %) had local pain or low grade sepsis, which respond to analgesics

and appropriate antibiotics. 1/15 (6.7 %) had respiratory depression wich required the next day of the puncture of the anterior abdominal. The gastrostomy was preserved and had

a function. And one of 15 patients had the presence of signs of peritonitis which necessitated PEG tube removal. Median duration of PEG was 135 days (range, 58–230).

Conclusion. One of the methods of the nutritional rehabilitation in patients with STS of the head and neck is application feeding via gastrostomy. PEG can be performed both on

stages of intensive care in patients with malnutrition and with a preventive strategies, for example, before start of radiation therapy. PEG is an established route for providing long

term enteral nutrition. This procedure is associated with frequent minor morbidity and with low risk of complications.

A B S T R A C T N O . : O - 2 9 2

Interstitial brachytherapy for childhood soft tissue sarcomas:

long term disease outcome & late eff ects

Siddhartha Laskar, Nehal Khanna, Ajay Puri, Sajid Qureshi, Ashish Gulia, Tushar Vora, Bharat Rekhi, Seema Media,

Girish Chinnaswamy, Shashikant Juvekar, Subhash Desai, Yogesh Ghadi

Tata Memorial Hospital, Mumbai, India

Key words: soft tissue sarcoma, children, interstitial brachytherapy, outcomes

Introduction. Radiation therapy forms an important component in the multimodality management of soft tissue sarcomas in children. Interstitial Brachytherapy (BRT) can be very

eff ective in improving disease control & reducing long term toxicity.

Aim. To evaluate the clinical outcome & long term adverse eff ects of interstitial brachytherapy (BRT) for children with soft tissue sarcomas (STS).

Materials and methods. From September 1984 to Dec 2013, 84 children (median age 15 years, range 1 to 18) with STS who received BRT as part of loco-regional treatment were

included. There were 49 males and 35 females. Majority (74 %) had primary lesions. Synovial sarcoma (30 %) was the most common histological type, and 44 % had high-grade

lesions. Treatment included wide local excision and BRT with or without external beam radiotherapy (EBRT). Forty children (66 %) received BRT alone.

Results. After a median follow-up of 70 months, the local control (LC), disease-free survival (DFS), and overall survival (OS) were 85 %, 74 %, and 77 %, respectively. LC was superior

in patients with tumor size ≤ 5 cm versus > 5 cm (96 % vs. 78 %, P = 0.08), symptom duration 2 months (100 % vs. 81 %, P = 0.06), and Grade I versus Grade II versus Grade

III tumors (100 % vs. 95 % vs. 70 %, P = 0.02). Children receiving a combination of BRT and EBRT had comparable LC to those receiving BRT alone (76% vs. 89%, p=0.28). There was

no signifi cant diff erence in LC for patients receiving LDR versus HDR BRT (84 % vs. 95 %, P = 0.31, for BRT alone; and 73 % vs. 83 %, P = 0.60, for BRT + EBRT). Surgical wound healing

complications was seen in 8 % patients. Subcutaneous fi brosis (31 %) was the commonest late complication followed by distal limb edema (5 %), joint stiff ness (3.3 %), & bone

growth abnormality (1.6 %). There was no neuropathy, non-healing ulcer or second malignancy.

Conclusion. Interstitial BRT with or without EBRT result in excellent outcome in children with STS. Radical BRT alone, when used judiciously in select groups of children, results

in excellent local control and functional outcome with reduced treatment-related morbidity.

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A B S T R A C T N O . : O P - 3 4 0

Childhood non rhabdomyosarcoma soft tissue sarcomas pattern and outcome

Mohamed Fawzy Hasan

NCI, Cairo University

Key words: childhood, soft tissue sarcoma, outcome

Introduction. Non rhabdomyosarcoma soft tissue sarcomas represent a group of heterogenous malignancies with varying grade and outcome. Most of data and managment

experience are derived from cummulative experience in adult patients. Paucity of information in pediatric age group calls for extensive studies.

Aim. the present study aimed to describe the overall survival, event free survival, clinical features, and treatment response in Non-Rhabdomyosarcoma soft tissue sarcomas (NRSTS).

Materials and methods. clinical features and tumor characteristics data of all 66 NRSTS patients younger than 18 years was reviewed. All patients were treated at the National

Cancer Institute, Egypt between January 2008 and December 2013. Surgery was the mainstay of treatment, while chemotherapy was administered to 35 patients, and only

15 patients received radiotherapy

Results. Synovial sarcoma and fi bromatosis were the most frequent histotypes with a median age of diagnosis was at 11 years. Tumors were greater than 5 cm in 52 patients (79 %),

high grade in 34 (51.5 %), originated from extremity in 42 (63.6 %) and metastatic at presentation in 14 (21 %). Overall survival at 5-year was 59.3 % for whole series of patients,

67.8 % in patients who underwent complete resection versus 63.6 % in patients who had marginal resection, 31.4 % in un-resected patients, and 20.8 % in patients presented with

distant metastases. The Event free survival was 17 % for the entire group of study patients. Outcome was unsatisfactory in patients with high-grade tumors, non-extremity location

of primary tumor, high-risk stratifi cation and un-resected primary tumor. The rate of objective response to neo-adjuvant chemotherapy was 28 %. Highest rates of recurrence was

in tumors > 5 cm in diameter, high-grade with positive surgical margins and received no adjuvant radiotherapy

Conclusion. Wide surgical resection of primary tumor with negative margins continues to be the mainistry of therapy. Local control was improved by the addition of postoperative

RT to high-grade tumors with positive margins. Both adjuvant chemo- and radio-therapy should be directed to high-grade tumors specially if inadequate surgical margins

and in neoadjuvant settings when complete resection could be facilitated by these modalities.

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A B S T R A C T N O . : P P - 1 0 5

Retinoblastoma: multimodal management in cancer care hospitalManoj Kumar Behera1, Faiz Akram2, Samrat Dutta3

1MHCI, Delhi; 2SMH, Delhi; 3NBMC, Siligudi

Key words: retinoblastoma, visual preservation, external beam radiotherapy

Introduction. Retinoblastoma, a primitive neuroectodermal origin tumor, is the most common primary intraocular cancer of childhood. With early detection and eff ective treatment,

this tumot remains the most curable of all childhood cancers with a survival rate currently exceeding 90 %.

Aim. To study the clinical spectrum & treatment outcome and to analyze the impact of combined treatment on visual preservation.

Materials and methods. In this retrospective analysis, a total of 50 patients were studied from March 2005 to 2015. Evaluable 35 patients analyzed for presenting features; sites

of involvement, treatment received and stage wise visual preservation. Most common age of presentation was 2–5 years (40 %) and leucocoria was the commonest fi nding (75 %).

More than 50 % of eyes had stage III–IV disease. Treatment was multimodal except stage I & II in which patients received only external beam RT with dose of 45 Gy/25#/5weeks. Rest

patients received RT after chemotherapy with Vincristine, cisplatin, etoposide and cyclophosphamide D1–4, weekly regimen or postoperatively. Following EBRT and chemotherapy

response assessed by USG, ophthalmoscopy and EUA.

Results. With the median follow-up of 30 months, this study showed that overall visual preservation rate of 98 % in stage I & II, 60 % in stage III, 40 % in IV and 32 % in stage V. None

of the patients reported had signifi cant radiation or chemo induced toxicities.

Conclusion. Visual preservation is excellent in retinoblastoma with current treatment modalities. With the advent of neoadjuvant chemoradiation, treatment goal has now shifted

from surgery to visual preservation in most of patients. EBRT has a defi nite role as primary or adjuvant treatment in management of retinoblastoma.

A B S T R A C T N O . : P - 1 3 3

Inhalational ambulatory anesthesia with sevofl urane in children with retinoblastoma

L. Martynov, T. Ushakova, N. Matinyan, A. Saltanov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: retinoblastoma, ambulatory anesthesia, inhalational anesthesia, sevofl urane

Introduction. Retinoblastoma (RB) is the most common intraocular malignant tumor in children. RB may present in utero and in early childhood and is characterized by a high

degree of malignancy and invasiveness. In family cases, the risk of RB in subsequent children increases up to 50 %. Patients with a genetic form of retinoblastoma and their

relatives are at risk and should have regular ophtalmological checkups. Thus the early detection of this disease is drastically important and dynamic observation during treatment

should be performed routinely. In Pediatric Oncology and Hematology Institute each session of ophtalmological chekup includes: ophthalmoscopy, digital widefi eld retinal cam

(RetCam Shuttle) and ultrasound. Due to the age of the children during the session of the checkup deep sedation is required. The practice of previous years showed a low effi ciency

of any sedation schemes in these children. Inhalational mask anesthesia with nitrous oxide also did not provide satisfactory conditions for the checkup. Currently, sessions

of ophtalmological evaluation are conducted ambulatory under mask inhalational anesthesia with sevofl urane.

Aim. To evaluate the eff ectiveness of the use of modern anesthetic sevofl urane during ophtalmological examination in ambulatory patients with RB, to study the spectrum of side

eff ects and complications of inhalational anesthesia with sevofl urane.

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Materials and methods. During the period from November 2014 to January 2016 185 ophtalmological checkups were performed under inhalational anesthesia with sevofl urane.

Exclusion criteria were: cardiac pathology, diseases of the respiratory system, including infectious diseases, signifi cant laboratory abnormalities. Monitoring in all cases comprised

of non-invasive measurement of blood pressure, ECG, pulse oximetry. Mask sevofl urane anesthesia was performed in VIMA (Volatile induction and Maintenance Anesthesia) mode.

Induction of anesthesia was performed on spontaneous breathing in the presence of a parent with sevofl urane inhalation up to 8 % and O2 6 l/min with pre-fi lled loop. After reaching

the MAC of loss of consciousness maintaining anesthesia was performed by inhalation of sevofl urane up to 3.5 %, O2 100 % 3.5 l/min. Ophthalmoscopy, digital microphotography

of the retina and other parts of the eye on RetCam Shuttle and ultrasound were performed. 2 minutes before the end of the diagnostic procedures the fl ow sevofl urane was stopped.

The child was transferred to the recovery room under the supervision of medical staff . Immediately after the checkup and 1 hour later the frequency and severity of the nausea,

vomiting, anxiety and respiratory failure were evaluated.

Results. The average duration of the procedure was 14 ± 6 minutes. The duration of induction was 45 ± 15 seconds (until loss of consciousness MAC). Mean BP was 59 ± 12 mm Hg.

There were no cases of hemodynamic instability during anesthesia. Wake-up time after the anesthesia was 5–11 min. The incidence of nausea after the procedure was 2.1 % (4 cases).

The frequency of the anxiety was 3.2 % (6 cases), propofol was administered in dose of 1 mg/kg. None of the patients did not require the extension of stay in the hospital due to

complications of anesthesia.

Conclusion. The study demonstrated that ambulatory ophtalmologic checkup in patients with RB under inhalational VIMA anesthesia with sevofl urane is a safe technique associated

with minimal side eff ect and provides rapid awakening and discharge of pediatric patient.

A B S T R A C T N O . : O - 2 0 1

Intraarterial and intravitreal chemotherapy in the combined treatment in children

with group C and D intraocular retinoblastoma

T. Ushakova1, O. Gorovtsova1, L. Martynov1, N. Matinyan1, I. Trofi mov1, A. Yarovoy2, O. Krivovyaz2,

S. Saakyan3, O. Ivanova3, B. Dolgushin1, V. Polyakov1

1Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia; 2S.N. Fyodorov Eye Microsurgery Complex; 3Moscow Helmholtz Research Institute of Eye Diseases, Russia

Key words: retinoblastoma, eye-saving treatment, selective intraarterial and intravitreal chemotherapy

Introduction. Among patients with intraocular retinoblastoma (IORB) there is a group of patients in whom the use of systemic chemotherapy (SCT), external beam radiotherapy

(EBRT) and methods of local destruction does not allow to achieve satisfactory outcome.

Aim. To expand the possibilities of eye-saving treatment in children with IORB by applying the local chemotherapy (LCT) while avoiding the use of EBRT. LCT comprises of selective

intraarterial chemotherapy (SIAC) in the ophthalmic artery and intravitreal chemotherapy (IVC). To evaluate the rate of the complications.

Materials and methods. The study comprised 42 patients (46 eyes) with primary IORB group C (18 eyes) and group D (28 eyes). In all cases patients underwent a series

of SCT (vincristine, etoposide and carboplatin) with addition of LCT. In 38 patients (42 eyes) LCT was performed after 2 courses of SCT. IVC was performed 106 times for 36 eyes

(median number 3 of procedures/eye), using melphalan in a dose of 16–20 mcg and/or topotecan in a dose 20mcg. SIAC was performed in 74 cases on 41 eyes (median number

2 of procedures/eye), using melphalan in a dose of 5.0–7.5 mg/m2. SCT was stopped as soon as stabilization was achieved and the following SIAC procedures were performed

with increasing doses of melphalan up to 7.5 mg (1 case), 2 patients were treated with 7.5 mg of melphalan and 1 mg of topotecan. In IVC melphalan doses was also increased up to

20 mcg (6 patients, 9 procedures), and in 7 patients IVC procedures with melphalan (20 mcg, 22 procedures) alternated with IVC procedures with topotecan (20 mcg, 17 procedures).

Methods of local destruction (brachytherapy, cryoablation, thermotherapy) were performed on 31 eyes.

Results. 17 (16 %) of the 106 IVC procedures were complicated by the development of chorioretinal atrophy in diff erent degrees of severity. 2 cases of SIAC were complicated

by intraoperative acute ischemic stroke and required prolonged rehabilitation. 8 cases of SIAC were complicated by intraoperative vascular collapse, treated successfully during

the procedure. Eye salvage rate in C group – 14 of 18 (13 of them – without EBRT), in D group – 25 of 28 (24 of them – without EBRT). All the children survived, the average

observation period was 17 months.

Conclusion. The study demonstrated that the rate of complications of LCT decreases with increasing experience of their conduct. Application of combined chemotherapy protocol

allowed to salvage the eye in 91.6 % cases without the use EBRT and in some cases without the use of methods of local destruction.

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A B S T R A C T N O . : P P - 2 2 0

Treatment outcome of retinoblastoma with combined systemic, intraarterial,

and intravitreal chemotherapy: a single center experience

Seung Min Hahn, Ju Yeon Lim, Won kee Ahn, Jung Woo Han, Dong Joon Kim, Sung Chul Lee, Chuhl Joo Lyu

Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea

Key words: retinoblastoma, chemotherapy, intraarterial injections, intravitreal injections

Introduction. In Korea, children who were diagnosed with retinoblastoma between 2001 and 2010 showed a 5-year survival rate of 95.5 %. However, the recent trend of treatment

for retinoblastoma is focused not only in increasing survival rate but also in saving the eye, and minimizing complications following the treatment. For this purpose, intraarterial

chemotherapy (IAC) and intravitreal chemotherapy became current standard treatment of retinoblastoma. From 2011, for the fi rst time in Korea, our hospital started IAC for

retinoblastoma. Intravitreal chemotherapy was introduced in 2013. To minimize the risk of relapse and metastasis to the central nervous system in advanced stage retinoblastoma,

we are using combined systemic, intraarterial, and intravitreal chemotherapy strategy.

Aim. To fi nd the best treatment protocol for retinoblastoma, we analyzed the treatment outcome of combined chemotherapy for retinoblastoma.

Materials and methods. From 2011 to 2015, 26 eyes of 25 patients (one bilateral case) received total 121 times of IAC in our hospital. Among them, 15 eyes of 14 patients were

newly diagnosed retinoblastoma who received alternate chemotherapy using IAC and systemic chemotherapy (Primary treatment group), the rest of 11 eyes were treated with IAC

after certain courses of systemic chemotherapy for salvage aim to save the eye (Secondary treatment group). Intravitreal chemotherapy was applied to 9 patients with vitreous

seeding. Patients’ demographic data were collected and eye salvage rate was assessed by the eye preservation time which was defi ned as the duration from the diagnosis to the time

of enucleation. Enucleations, death of the patients were defi ned as events of the eyes.

Results. Total 15 eyes of primary treatment group were classifi ed according to the International Classifi cation of Retinoblastoma (ICRB) as group B (n = 1), group C (n = 2), group

D (n = 5), or group E (n = 7). Secondary treatment group eyes were classifi ed as group D (n = 6), or group E (n = 4). Two to nine times of IAC (median 4.5 times) and fi ve to twenty

nine courses (median 10.5 courses) of systemic chemotherapy were performed in the patients. Two to six courses (median 4 courses) of intravitreal chemotherapy was performed.

Vincristine, carboplatin, etoposide, and cyclosporine chemotherapy was most commonly used regimen for systemic chemotherapy, and melphalan was used for intraarterial

and intravitreal chemotherapy. During the median follow-up period of 35.8 months (range, 8.6 to 59.1 months), overall eye salvage rate was 44.8 ± 11.9 %. The eye salvage rate of

each treatment group was 58.7 ± 14.6 % for primary treatment group, and 28.0 ± 16.4 % for secondary treatment group. Among 26 eyes, 10 eyes resulted in enucleation, and one

patient expired due to secondary juvenile myelomonocytic leukemia. The combined treatment was tolerable without signifi cant complications to most patients.

Conclusion. There were limitations of the study to evaluate the exact effi cacy of each treatment because applications of IAC, and intravitreal chemotherapy have been changed

through years. Recent active treatment of primary IAC and intravitreal chemotherapy have reduced the number of systemic chemotherapy to the patients. The result of combined

treatment for retinoblastoma was favorable and tolerable for the patients.

A B S T R A C T N O . : O P - 3 1 2

Expression of transcriptional target of p53 protein in human retinoblastoma

Lata Singh1, Seema Kashyap1, Neeru Saini2, Neelam Pushker1, Seema Sen1, Anjana Sharma1 1All India Institute of Medical Sciences; 2Institute of Genomics and Integrative Biology

Key words: retinoblastoma, p53, Bcl-2 protein, immunohistochemistry, RT-PCR

Introduction. Deregulation of apoptotic pathway leads to abnormal development, proliferation, and treatment resistance in cancer. Bcl-2 protein family play a key role in the control

of apoptosis and in the initiation of the apoptotic pathways. Noxa and Puma are transcriptional targets of p53 which play an active role in p53-induced apoptosis.

Aim. The aim of our study was to evaluate the expression of PUMA, p53 and NOXA and its prognostic signifi cance in human retinoblastoma (Rb).

Materials and methods. Prospective analyses of 60 primary enucleated retinoblastoma specimens were immunohistochemically assessed for PUMA, p53 and NOXA expression

and then confi rmed by western blotting. RT-PCR was also performed to amplify these genes. Cytoplasmic staining was considered as positive and the expression was correlated with

clinical parameters, tumor diff erentiation and histopathological high risk factors like massive choroidal invasion, scleral invasion, optic nerve invasions etc.

Results. There were total of 45 (75 %) cases with poorly diff erentiated retinoblastoma. Necrosis and calcifi cation was found to be present in 37 and 17 cases respectively.

Of the 60 eyes, 20 (33.3 %) had massive choroidal invasion, 17 (28.3 %) had retrolaminar and cut end optic nerve invasion, 9 (15 %) had iris & ciliary body invasion and 5 (8.33 %)

had anterior chamber invasion. Expression of PUMA was observed in 26/60 (43.33 %) cases, p53 expressed in 22/55 (40 %) cases and NOXA was positive in 28/60 (46.66 %) cases.

These were confi rmed by western blotting and RT-PCR technique. p53 alone signifi cantly correlated with poor tumor diff erentiation. Similarly, PUMA and NOXA correlated with tumor

diff erentiation and various histopathological high risk factors.

Conclusion. This study showed that expression of PUMA and NOXA might be regulated by p53 tumor suppressor protein. Therefore, these proteins may be used as a future therapeutic

target in combating retinoblastoma. Future investigation of Bcl-2 pathway might be helpful for developing biomarkers in the management of retinoblastoma patients.

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A B S T R A C T N O . : O - 3 4 7

The eff ectiveness of ophthalmological treatment of recurrent

and resistant retinoblastoma

A. Yarovoy1, T. Ushakova2, V. Polyakov2, O. Golubeva1, O. Krivovyaz1, O. Gorovtsova2

1The S.N. Fyodorov Eye Mircosurgery Federal State Institution, Moscow, Russia;2Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: local treatment, brachytherapy, laser

Introduction. Presently chemotherapy (CT) is recognized as the fi rst line treatment for retinoblastoma (Rbl). However, signifi cant number of cases of CT insuffi ciency requires

additional local treatment – cryotherapy, laser thermotherapy, or brachytherapy. There is limited information concerning the eff ectiveness of these methods after completing

the CT with the lack of eff ectiveness.

Aim. to evaluate the results of the local treatment in the cases of insuffi cient retinoblastoma response to chemotherapy

Materials and methods. A total of 388 tumors in 102 eyes of 82 children with insuffi cient response to systemic and local CT were included into the study. Bilateral retinoblastoma

was in 63children, 19 children were monocular. Twenty fi ve eyes were group A, 33 – group B, 23 – group C, 21 – group D.

One hundred and eight tumors were treated with brachytherapy (Ru-106, Sr-90). In 28 eyes the plaques were relocated successively to irradiate two or three tumors. Two hundred

sixty-seven tumors were treated with laser thermotherapy, 56 with cryotherapy. Ten eyes with resistant recurrent multiple Rbl were treated with the focal therapies simultaneously

with intra vitreous melphalan injections (16–20 μg).The follow-up is from 3 to 60 months (mean, 19).

Results. Ninety seven eyes (95 %) were retained. The regression pattern types after each of the treatment methods are evaluated. Complete or partial regression was achieved

in 90 tumors (83 %) after brachytherapy, in 203 tumors (76 %) after thermotherapy, in 49 tumors (87.5 %) after cryotherapy. There were 56 (21 %) recurrences after thermotherapy,

4 (7 %) after cryotherapy. There were no cases of metastases.

Conclusion. Local treatment is a necessary and eff ective part of Rbl treatment especially in chemotherapy insuffi ciency. Simultaneous intra vitreous CT seems to increase the eff ect

of focal therapy.

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A B S T R A C T N O . : O P - 1 3 7

Multimodality treatment outcome of hepatoblastoma:

our experience in a resource limited country

ATM Atikur Rahman, Mehnaz Akter, Momena Begum, Chowdhury Shamsul Hoque Kibria, Afi qul Islam

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Key words: hepatoblastoma, multimodality treatment, outcome

Introduction. Hepatoblastoma is the most common primary hepatic malignancy in childhood, accounting 0.8–2 % of all paediatric cancers. The incidence has been an increasing

over the last two decades but the aetiology for it still remain unclear. Predominantly seen in premature and/or very low birth weight children. Significant improvements in multi-

modality treatments have been achieved over the last two decades. Five-year overall survival (OS) rates now approaching 70–75 %. The purpose of this paper is to report on about

the experience in management of hepatoblastoma in a tertiary centre in an developing country like Bangladesh.

Aim. To review the records of management of hepatoblastoma in the Paediatric Haematology and Oncology department of Bangabandhu Sheikh Mujib Medical University (BSMMU)

over 8 yrs from January 2004 to December 2012.

Materials and methods. A retrospective analysis has been done in BSMMU with children having hepatoblastoma through January 2004 to December 2012. Patients demographics,

mode of presentation, method of diagnosis, extent of tumor at diagnosis,surgical procedures performed, complications of treatment and outcome were compiled by reviewing

medical histories, radiology, pathology, and surgery reports. Based on imaging using CT scan or MRI, all patients were assigned a PRETEXT stage and four groups of patients were

identifi ed as PRETEXT I–IV.

Patients were treated with neoadjuvant therapy (chemotherapy followed by surgery and then again chemotherapy). Cisplatin and Adriamycin as in the PLADO (PLA, Platinum group;

DO, Doxorubicin) regimen was administered to all patients every 21 days interval. Every cycle contains PLA on day-1 at a dose of 80 mg/m2 for continuous 24-hr intravenous infusion

and DO at a dose of 30 mg/m2/day over 48-hr continuous intravenous infusion on days 2 and 3.

Patients were routinely reassessed after three cycles for surgical resection. If the tumor was found to be inoperable, one more cycle of chemotherapy has been given to them. Surgical

decision had been taken by the pediatric surgical oncology team. Postoperatively, 2 to 3 cycles of chemotherapy were given to a total of 6 cycles. The patients were followed after

treatment according to the institution’s protocol with serial AFP levels and radio-imaging.

Results. In this study population median age of was 12 months (3–60 months) and male:female was 3.3:1. Nine patients were treated with neoadjuvant chemotherapy incorporating

cisplatin and adriamycin. Primary surgery was done in four patients. Extent of hepatic resection in the operated patients varied. Mixed type was the predominant histopathological

diagnosis. Adjuvant chemotherapy was well tolerated with no morbidity or mortality. Five-year event-free survival (EFS) and overall survival (OS) of all the 13 patients is 76.9 %.

All the nine patients who could complete multimodality treatment are alive with no evidence of disease or complications with median follow-up of 63 months (46–122 months).

On the basis of staging (PRETEXT), the 5-year OS is as follows: PRETEXT-I 100 %, PRETEXT-II 100 %, PRETEXT-III 66.7 % and PRETEXT-IV 0 %.

Conclusion. Treatment of hepatoblastomas with multidisciplinary approach were well tolerated. OS and EFS of all patients were comparable with published studies.

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A B S T R A C T N O . : P - 1 4 4

Surgical options in complex treatment of focal liver lesions in pediatric patients

E.F. Kim1, A.V. Filin1, A.V. Semenkov1, D.S. Burmistrov1, O.V. Dimova1, T.N. Galjan1, E.U. Krjijanovskaia1,

A.V. Metelin1, S.R. Varfolomeeva2, D.Yu. Kachanov2, T.V. Shamanskaya2, R.A. Moiseenko2, A.V. Petrushin2

1Russian Scientifi c Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia;2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: focal liver lesions, hepatoblastoma, anatomical resection, liver transplantation, pediatric

Introduction. Hepatic tumors account for 1–4 % of the solid tumors in children.

Aim. Results assessment of surgery treatment for pediatric liver tumors.

Materials and methods. From April 2008 to December 2015, 81 pediatric patients (3 months – 17 years old) with a variety of focal liver lesions underwent surgery at Petrovskiy

National Research Centre for Surgery. Malignancies (75.3 %) were presented by hepatoblastoma (66.7 %), undiff erentiated (embryonal) sarcoma (3.7 %), hepatocellular carcinoma

(2.5 %), malignant rhabdoid tumor (1.2 %) and rhabdomyosarcoma (1.2 %). Benign lesions or neoplasms (22.2 %) were presented by focal nodular hyperplasia (7.4 %), mesenhymal

hamartoma (4.9 %), hepatocellular adenoma (2.5 %), infantile hemangioendothelioma (2.5 %), benign teratoma (2.5 %), cavernous hemangioma (1.2 %), infl ammatory

pseudotumor (1.2 %). Two patients (2.5 %) had a parasitic liver disease: cystic and alveolar echinococcosis.

Results. Surgical treatment consisted of anatomical resection (lobe- and extended lobectomies – 82.5 %, segmentectomies – 4.9 %, and living-donor liver transplantations – 12.4 %.

In one case of cystic echinococcossis was made total pericystectomy. In groups of benign and parasitic diseases there were no deaths. The treatment’s results for patients with the

hepatoblastoma are presented below. The age of patients in this group ranged from 3 months to 17 years (median – 19.13; 25th and 75th quartiles – 10.53– 34.1 months). In 87 %

of cases, the age did not exceed 4 years old. Patients classifi ed by system PRETEXT: I – 2 % (n = 1), II – 39 % (n = 21), III – 31 % (n = 17), IV – 28 % (n = 15). In 13 children (24 %)

detected lung metastases. In 2 cases hepatoblastoma developed on the background of Beckwith–Wiedemann syndrome. A biopsy of the tumor before treatment was performed in

31 (57.4 %) patients. Neoadjuvant chemotherapy performed in 54 cases (96 %). Tumor regression with reduction of the tumor stage were achieved in 7 patients (13.5 %). Progression

of tumor and stage of the disease were found in 4 cases (7.7 %). Lung surgery due to the remote metastases required in 6 cases (11.4 %). In 89 % (n = 48) of patients performed

anatomical resection of the liver and in 11 % (n = 6) – living related liver transplantation. Extended hemihepatectomies and bilateral liver resections were performed in 65 %

(n = 35). All resection performed without Pringle maneuver. The volume of blood loss in patients with resection was 17.02 ± 14.67 ml/kg. Hospital mortality rate was 1.85 %

(n = 1). Three patients (5.6 %) died for the fi rst year after surgery. The causes of death were complications of adjuvant chemotherapy (n = 2) and pulmonary embolism during surgery

for tumor recurrence (n = 1). One patient died after 15 months due to the progression of distant metastases in the lungs. The actuarial survival rate (overall/disease-free) for patients

with hepatoblastoma was, respectively: 1 year 0.94 ± 0.03/0.84 ± 0.05; 3 years 0.89 ± 0.04/0.84 ± 0.05; 5 years 0.89 ± 0.04/0.84 ± 0.05; 7 years 0.89 ± 0.04/0.84 ± 0.05. In patients

after resection the same results were higher: 1 year 0.96 ± 0.03/0.88 ± 0.05; 3 years 0.90 ± 0.05/0.86 ± 0.05; 5 years 0.90 ± 0.05/0.86 ± 0.05; 7 years 0.90 ± 0.05/0.86 ± 0.05.

Conclusion. Surgery is the preferred treatment of benign and malignant liver lesions in pediatric patients. Long-term results with high survival rates for combined treatment

of hepatoblastoma demonstrate broad opportunities both resection and liver transplantation in cases of selection of optimal surgery plan and chemotherapy protocols.

A B S T R A C T N O . : O P - 2 1 2

Clinical experience and outcome of hepatoblastoma in children:

a 9-year retrospective study

Sanjeev Khera, Amita Trehan, KLN Rao, Deepak Bansal, Nandita Kakkar, Radhika Srinivasan

Postgraduate Institute of Medical Education & Research, Chandigarh, India

Key words: hepatoblastoma, outcome, high risk

Introduction. Hepatoblastoma (HBL) is the most common primary liver tumor in children. Metastatic disease at presentation, alpha-fetoprotein (AFP) less than 100 ng/ml, PRETEXT

IV, undiff erentiated histology are usual poor prognostic markers. Neoadjuvant chemotherapy with surgical resection has led to increased survival of these patients.

Aim. This single centre study assesses the outcome and the factors aff ecting prognosis of children treated as per guidelines of SIOPEL 3 in a low middle income country (LMIC).

Materials and methods. Forty children with HBL treated as per protocol from Jan 2007 to Dec 2015 were analyzed. The diagnosis was established by imaging, AFP and histology /

cytology.

Results. 27 boys & 13 girls; median age 12 months (2–120) with a mean symptom diagnosis interval (SDI) of 5.3 weeks (1–24) were diagnosed. Multifocal involvement was seen

in 7/40. Median AFP at diagnosis: 16 500 ng/ml (3–406198). 2 had AFP less than 100 ng/ml. Mean platelet count at diagnosis 701375/cmm (95 % CI 595510–807239). 7 (17.5 %)

had metastatic disease (6 lungs; 1 adrenal). PRETEXT: I–IV: 6, 13, 11 & 9. Nineteen: high risk (HR) disease (7 with SDI more than 8 weeks).

13/40 defaulted/refused therapy. All cases received neoadjuvant chemotherapy. 3 deaths occurred prior to surgery. 25 underwent surgery; complete resection (CR): 18/25

(14: well; 3 relapsed, 1 died). 7 cases subtotal resection: 2: well, 4 had persistant disease and 1 death. 2/3 children who received salvage chemotherapy with Docetaxel had no response.

HR patients had a poorer outcome (P = 0.012), more multifocal disease (P = 0.0395) and signifi cantly lower CR (P = 0.0016) as compared to standard risk patients. On comparing

well Vs relapsed/residual disease cases; there was no diff erence in AFP levels, volume reduction after neoadjuvant therapy, metastasis, & histopathology (P = ns). Relapsed/residual

disease was signifi cantly higher in those with higher mean age (24 mth Vs 12 mth); PRETEXT IV & multifocal disease (P = 0.02 & 0.04). 16 of 27 patients are presently well; median

follow up being 48.3 months (7–101).

Conclusion. 59 % (16/27) of cases who were treated are well. Higher age at presentation, multifocality and high PRETEXT was associated with poor outcome. HR patients had

a longer SDI. Docetaxel as a salvage therapy was not eff ective. Liver transplant, not available at our centre, would have helped the children with incomplete resection.Delayed referral

and diagnosis remains a problem in LMIC’s as evident by greater SDI in those with HR disease.

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A B S T R A C T N O . : P - 3 2 1

Assessment of early tumor response to cisplatin monotherapy

in patients with standard risk hepatoblastoma

D.Yu. Kachanov1, E.V. Pheoktistova1, T.V. Shamanskaya1, R.A. Moiseenko1, A.A. Merishavyan1, M.A. Tarasov1,

G.V. Tereshchenko1, S.R. Talypov1, E.F. Kim2, D.S. Burmistrov2, A.V. Filin2, S.R. Varfolomeeva1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Scientifi c Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia

Key words: hepatoblastoma, children

Introduction. Hepatoblastoma is the most common primary malignant liver tumor in children. Implementation of protocols for risk-adapted therapy may de-escalate therapy in the

group of standard risk patients with preservation of the eff ectiveness of treatment.

Aim. To assess the dynamics of tumor response to cisplatin monotherapy in the group of standard risk patients with hepatoblastoma treated in the Federal Scientifi c and Research

Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev.

Materials and methods. The study included 21 patients of standard risk hepatoblastoma treated during the period 01.2012–09.2015 (45 months). The diagnosis was based on

histological examination. Staging was according to the PRETEXT system. Patients were treated according to SIOPEL-3 SR protocol, including single-agent cisplatin (4 courses of

neoadjuvant therapy and 2 courses of adjuvant therapy). Assessment of the level of alpha-fetoprotein (AFP) and volume of tumor (cm3) was done at diagnosis and after the 2nd course

of chemotherapy.

Results. 16 patients were included in the assessment of the dynamics of the response. Male to female ratio was 0.6:1. The median age at diagnosis was 6.3 months

(range 0.1–36.9 months). The distribution for PRETEXT stages: stage I – 2 (12.5 %) patient; II – 12 (75.0 %), III – 2 (12.5 %).

The median AFP level at diagnosis (n = 16) was 329.741 (range 847–1.971.991), after 2 courses of cisplatin (n = 16) – 20.229 (range 211–181.400) (P = 0.0006). The decrease

in AFP levels of ≥ 1 log was observed in 11 (68.7 %) patients, less than 1 log – in 4 (25.0 %) patients, increase in the level of AFP is detected in 1 (6.3 %) case. Estimation of the volume

of the tumor at diagnosis (n = 16): median 332 cm3 (range 180-970), after 2 cycles of chemotherapy (n = 16): median 230 cm3 (range 15–800) (P = 0.001). All patients continued

scheduled therapy. The patient with increasing values of the AFP was transferred to the intensifi ed therapy according to the SIOPEL-4 protocol.

The median duration of follow-up was 18.7 months (range 5.8–36.2 months). All 16 patients are alive without any events.

Conclusion. The fi ndings suggest that cisplatin as monotherapy is eff ective in the treatment of standard risk hepatoblastoma. The decrease in the level of AFP is less than 1 log after

2 courses of treatment with cisplatin should not be considered as a criterion of intensifi cation therapy.

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A B S T R A C T N O . : P - 3 8 3

Characterization of germinals pediatric tumors in National Institute of Oncology

and Radiobiology of Cuba (2000–2012)

Migdalia Perez, Mariuska Forteza, Jose Alert, Jesus Reno

National institute of Oncology and Radiobiology of Cuba, Havana

Key words: tumors, children, germ cells

Introduction. The tumors of germ cells in infancy constitute a heterogeneous group with diverse histology, represent of the 2 % the 5 % of the set of malignant diseases of

infancy. The histological and genetics properties of these tumors vary according to the place of the primary tumor, the kind and the age of the patient. His clinical and histological

characteristics diff er signifi cantly from the ones that predominate in adult age with a range of survival to 5 years in the teens of 15 to 19 years of 90 % at localized and of 60 %

in patients with advanced tumors, if they are driven with surgery and Chemotherapy adyuvante developed in the last ones two decennia, which has been able to control these tumors

in very aggressive occasions, with tall potential of metastización.

Aim. Describing the clinics characteristic, histology, treatment received in studied patients and estimating functions of global survival, free survival of progression and the infl uence

of some variables in the same.

Materials and methods. In Pediatríc Service of Oncology and Radiobiology National Institute of Cuba, Himself I accomplish a retrospective study from January 1 the 2000

to December 31 , 2013 ,of patients with diagnosis of germinal tumors the, total of patients sign got constituted for 16 patients .

For the execution of investigation they checked the clinical histories of all of the patients included in the study. A data base in SPSS elaborated 12 itself. The variables analyzed

intervening the statistical method of Kaplan Meier themselves and results were exposed in draw and graphics.

Results. This entity’s prevalence was bigger in the female sex with a 69 %. The 46 % of the patients were on the age bracket of 6 to 10 years followed by the 39 % of 16 to 20 years.

The incidence in our study was superior in patients of 6 to 10 years (46 %).

Generally they show the tumors of germ cells like indolent masses. 50 % of our patients I present pain like initial symptom and the bigger their number corresponded to the female

sex, the 43 % showed up with tumor like initial sign, and the bigger percent went from the masculine sex their, I grasped lender of securities in the revised bibliography. More frequent

histology’s were the Seno Endodermis’s tumor in the fi rst place 46 %, followed of the Teratoma Inmature with 23 %. The 75 % was located at ovary in our cases and their more frequent

histology was the Teratoma Inmature.

Conclusion. With present-day regimens of poly-chemotherapy correlated to surgery and at times we have achieved bigger number of complete remissions lifting the índices

of survival of our patients.

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A B S T R A C T N O . : O P - 0 7 8

Malignant melanoma in children: etiology, treatment, and prognosis

Ali Varan, Ozay Gokoz, Figen Ozgur, Burca Aydin, Bilgehan Yalcin, Nilgun Kurucu, Tezer Kutluk, Canan Akyuz

Hacettepe University Cancer Institute, Ankara, Turkey

Key words: malignant melanoma, prognosis, treatment

Introduction. Primary malignant skin tumors are very rare in children. Most common skin tumor in children is malignant melanoma as in adult population. SEER reported the

incidence of malignant melanoma in children to be 1.3/1 million. Several underlying pathological conditions like giant cell nevi, xeroderma pigmentosum, radiotherapy, some

immunodefi ciency disorders have been described in cutaneous malignant tumors. The survival rate is over 80 % while this ratio is lower when there is regional and distant metastasis.

Overall survival rates in regional and distant metastasis were 60 and 25 %, respectively. Surgery, chemotherapy, and targeted therapy were used for the treatment of the patients.

Aim. The aim of this study is to evaluate the etiology, treatment and prognosis of the malignant melanoma in children.

Materials and methods. Twenty-one patients who had been diagnosed with malignant melanoma between 1972 and 2015 were retrospectively analyzed. Staging was done

according to AJCC. High dose interferon, or CDDP based regimens have been used. Mean and median values were used for demographic characteristics. Kaplan–Meier survival curves

were used for survival analysis. The patient groups were compared in terms of survival duration using a log-rank test.

Results. We could defi ne the etiologic factors in only 5 (23.8 %) patients. In three patients malignant melanoma occurred within the area of Giant hairy cell nevus, one melanoma

patient previously had bone marrow transplantation due to Gricelli syndrome, one patient also had Xeroderma pigmentosum. Tumors were located in head and neck (7), trunk (5),

ocular (2), upper extremity (2), lower extremity (2), and intracranial (1). Tumor location was not detected in two patients. Stage distributions were 0 (1), I (1), II (5), III (6), and IV (6).

The stage was not defi ned in two patients. Six patients did not require treatment due to low stages. High dose interferon was given to 9 patients. Three patients were treated with

cisplatin based protocols, and three with other chemotherapeutic regimens. Five patients died with disease progression. Overall survival was 75 %, whereas event-free survival rate

was 70 %. Prognostic factors on survival were treatment with interferon (P = 0.001) and metastatic disease (P = 0.01).

Conclusion. Malignant melanoma is rare tumor in childhood. Metastasis is the worst prognostic factor. Melanoma patients were treated successfully with high dose interferon in

our series.

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A B S T R A C T N O . : P - 1 1 7

Own experience of treatment of aggressive fi bromatosis at children

V.B. Makhonin1, R.R. Bayramgulov1, 2, A.A. Gumerov2

1Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: desmoid, fi bromatosis

Introduction. Aggressive fi bromatosis (AF) (or desmoid fi broma) is a special variant of mesenchymal tumours of soft tissues, taking the place between non-malignant and

malignant tumours. AF in contrast of malignant tumours doesn’t have the possibility for metastasis forming but can infi ltrate and relapse multiple times after surgical removing.

Because of relapses and presence of non-resectable forms of AF, this disease requires complex approach of treatment. Despite of messages on possibility of usage of diff erent schemes

of chemotherapy, radiation and hormonal treatment, the real possibility of estimation of eff ectiveness of these methods is hard because of small numbers of observations.

Aim. To perform the analysis of own results of treatment of patients with AF during 14 years.

Materials and methods. During the period of 2002–2015 it were treated 17 patients with AF at age 4 months to 16 years old in our center. The most frequent aff ected area were

nates (9 patients). In all cases the fi rst line of therapy was surgical treatment; macroscopical full resection was registered in 12 of 17 cases, biopsy only was done in 1 case. Five patients

(older 1 year, total resection) in remission now with the follow-up from 6 to 11 years. Remission in these cases was reached only after surgical treatment and in 12 cases, adjuvant

therapy was used. Applied scheme of chemotherapy: vinblastine and methotrexate 10 weeks – at 9 patients, and at 5 patients relapse was diagnosed. This situation requires repeated

surgery and usage of the other scheme of therapy (VAC and etc.); radiation therapy was used at 3 children (total lesion dose – 44 Gy). Five children received prolonged therapy with

vinblastine and methotrexate in combination with tamoxifen – 3 of them. Prolonged therapy duration was 2 years.

Results. As a result of treatment, remission was reached at 3 children (during 7 years), remission – 6 children (in terms from 3 to 9 years), late relapse – 2 children

(in terms 3 and 5 years from treatment end).

Conclusion. 1. In case of possibility of radical surgery, the fi rst line of therapy is resection of tumour. 2. In case of non-full resection, non-resectable tumours, in case of relapses (even

in case of total resection of relapsed tumour) the adjuvant therapy is indicated. 3. Schemes with prolonged (from 6 months) usage of vinblastine and methotrexate (in several cases,

especially at boys, tamoxifen can be used) is preferred. Usage of other medications and radiation therapy must be considered as second-line treatment.

A B S T R A C T N O . : O P - 1 7 6

Subcutaneous Ewing sarcoma: an indolent course of disease E.S. Tyutikova, D.V. Litvinov, N.P. Makarova, V.Yu. Roshchin, A.P. Shcherbakov, N.V. Zhukov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: a rare case, subcutaneous Ewing sarcoma, soft tissue sarcoma, oncology

Introduction. Ewing sarcoma is a highly malignant neuroectodermal tumour characterized by the presence of translocation with the involvement of EWS gene. Radical resection

of tumour and modern aggressive chemotherapy programs make it possible to cure most of the patients, however without treatment all the patients die of disease progression within

a short time. Most frequently in case of Ewing sarcoma, the primary site is located in bones, less frequently it occurs in the depth of soft tissue, extremely rarely – in the subcutaneous

tissue and skin. To date, treatment approaches for Ewing sarcoma have not varied according to the localization of primary site, however available data suggest a fundamentally

another prognosis for patients with superfi cially located primary site of the tumour. Yet a number of described cases of subcutaneous Ewing sarcoma is extremely limited. None of the

cases illustrated the natural history of the disease (without specifi c treatment).

Aim. Presentation of rare clinical setting.

Materials and methods. A fi fteen-year-old patient with unremarkable development, negative familial history, without concomitant diseases. In July 2012 in the inguinal fold

area on the right a painless mobile tumour-like mass up to 2 cm in diameter appeared. The total biopsy performed by a local pathologist showed no signs of malignant growth.

About 1.5 years later (11.2013) the mass growth up to 1.5 cm in diameter was detected again in the same area. After the excision biopsy performed by the local pathologist the patient

was diagnosed with dermal cylindroma (adenocyctic canser). The material was sent to the reference laboratory of our clinic, where on the revision of all biopsy materials the following

diagnosis was established: ES with high proliferative activity (Ki-67 > 80 %) and EWSR1 gene rearrangement in t(11;22). However, the patient discontinued surveillance and was

referred to our clinic only in September 2014 (after more than 2 years after initial biopsy). A detailed examination that included computer tomography, magnetic resonance imaging

and positron emission tomography revealed no abnormal foci except for 3 palpable mobile foci of 0.5–1.5 cm in size in the area of postoperative cicatrix. A total biopsy was performed

on these lesions and the presence of Ewing sarcoma in the lesions was confi rmed.

Results. Thus, in spite of high proliferative activity and the presence of proven genetic rearrangement typical for ES, the subcutaneously located tumor had clearly distinct clinical

course – the self-limitation of tumor mass volume (no more than 2 cm in size), the absence of distant metastases for more than 2 years. Despite this, the treatment according

to CWS-2009 protocol was initiated, however after 3 blocks of therapy, the patient refused further treatment. To date, the patient is alive without any signs of recurrence.

Conclusion. The given case is indicative of specifi c clinical course of subcutaneous Ewing sarcomas that probably require less aggressive treatment.

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A B S T R A C T N O . : P - 2 0 5

Congenital atypical teratoid/rhabdoid tumor of the brain, rhabdoid tumor of the kidney

with heart metastases in a 2-months-old girl. A case report

K. Rastoltsev1, R. Burenkov1, D. Lantsov2, N. Kishchenko2, S. Mamtchenko3, E. Tashirova3

1Kaluga Regional Children’s Hospital, Russia; 2Kaluga Regional Oncology Dispensary, Russia;3Morozov Children’s City Clinical Hospital, Moscow, Russia

Key words: atypical teratoid/rhabdoid tumor, AT/RT, rhabdoid tumor

Introduction. Atypical teratoid/rhabdoid tumor (AT/RT) and rhabdoid tumor of the kidney (RTK) are representatives of a malignant rhabdoid tumor family. AT/RT is a very rare,

malignant, embrional tumor of the central nervous system. According to WHO Сlassifi cation 2007 of tumours of the Сentral Nervous System, AT/RT belongs to embrional brain tumors,

grade IV. RTK according to WHO Classifi cation 2004 of Renal Tumors mainly belongs to mesenchymal tumors of the childhood. The part of AT/RT, according to various authors, consists

of 1–3 % of all CNS tumors of the childhood; with the frequency of diagnostic tests three of one million children. Synchronous primary AT/RT with RTK are extremely rare and occur

in 2–15 % of all cases AT/RT. A development of AT/RT connected with deletion and/or mutation in 22q11.2, where INI1 gene is located. Protein, encoded by this gene provides more

effi cient access to transcription unit and it is a biological, epigenetic supressor of the tumor’s growth with a complex mechanism of the biological activity.

Aim. We report a case of congenital AT/RT of the brain and rhabdoid tumor of the kidney with heart metastases.

Materials and methods. Patient G., hypotrophic girl at the age of two months and ten days. RT of the brain and the right kidney were diagnosed at her birth. Recommended

chemotherapy protocol “EUROHAB” was not started because of her poor condition. Death from multiple organ dysfunction syndrome at two moths and ten days. On autopsy a median

tumor was found, located supra- and infratentorial, measured 10 cm in its greatest dimension, with spreading to cerebellum lobes, superior vermis, brain stem, aqueduct, third

and fourth ventricles (with their full obliteration). In the right kidney a dense, white, encapsuled tumor was found, measuring 14 cm, taking up to 90 % of entire organ volume.

In lateral wall of left cardiac ventricle two dense, white, exofi t tumor nodules were found, 0.8 cm in size, spreading into miocardium by 0.3 cm. In liver, left kidney, both adrenal glands,

lymph nodes multiple metastases were found up to 5.5 cm in size.

Results. Hystopatologically, brain tumor was hypercellular, mainly presented by primitive basophilic neuroectodermal cells, which form primitive pseudorosettes and “glandular”

structures, solitary rhabdoid cells. Right kidney tumor along with metastases were presented by vast fi elds of rhabdoid cells with solitary multinuclear forms. Immunohistochemically,

tumor expressed Vim, CD56, however INI1 was absent. In kidney tumor and metastases additional expression of EMA, Syn was found.

Conclusion. AT/R, RTK with heart, liver, contralateral kidney and lymph nodes metastases, “atipical” expression primitive neuroendicrine cell antigenes (CD56, Syn).

A B S T R A C T N O . : O P - 2 0 9

Colorectal carcinoma in children and adolescents. Single center experience

Asmaa Mohammed Hamoda, Ahmed Ibrahim Elhemaly, Wael Zekri Khalid

National Cancer Institute Egypt, Cairo, Egypt

Key words: colorectal carcinoma

Introduction. Colorectal carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure

because of lack of data.

Aim. To evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome.

Materials and methods. Records of children who had colorectal carcinoma and were treated at our institute were reviewed retrospectively between 2008–2014. Information

recorded for each patient included age, sex, predisposing factors, positive family history, clinical characteristics, diagnostic procedures, extent of disease, treatment methods,

histological types, and outcome.

Results. There were 7 males and 8 females who were treated for colorectal carcinoma, their ages ranged from 13–17 years. Predisposing conditions were found in 2 patients, one with

familial adenomatous polyposis and another with neurofi bromatosis type 1.4 cases had positive family history with cancer colon. Predominant symptoms were picture of intestinal

obstruction in 9 cases, weight loss in all cases, rectal bleeding in 5 cases. Colon was the site for the primary tumor in 10 patients, while rectum was the primary site in 5 patients.

Histological type in all patients was mucinous adenocarcinoma (100 %). Surgical intervention was just endoscopic biopsy in 7 patients, complete resection was done in 8 patients.

All patients except one- who did complete surgical excision with absence of lymph node involvement – received adjuvant chemotherapy, and 1/5 of rectal adenocarcinoma received

rectal radiotherapy. 9 patients died of disease in a period ranging from 1 month to 1 year after initial surgery. 7/9 patients who died did just biopsy without complete resection

and the other 2 patients, 1 had NF1 and developed irresectable HGG and died out of progressive brain tumor and the other one had history of familial adenomatous polyposis

and died out of disease progression.

Conclusion. Delayed diagnosis, advanced stages of disease at presentation, delayed surgery and, most importantly, mucinous type of histology are the major determinants of poor

outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms

of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding.

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A B S T R A C T N O . : P P - 2 2 9

Pediatric infl ammatory myofi broblastic tumor: Experience of multicenter cooperation

D.Yu. Kachanov1, T.V. Shamanskaya1, A.M. Suleimanova1, S.R. Talypov1, N.N. Merkulov1, R.S. Oganesyan1,

N.G. Uskova1, M.V. Teleshova1, Yu.V. Olshanskaya1, A.N. Kazakova1, V.Yu. Roshchin1, D.M. Konovalov1,

D.S. Burmistrov2, A.V. Filin2, A.F. Matytsyn3, S.V. Kaplunov4, S.R. Varfolomeeva1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Russian Scientifi c Centre of Surgery named after Academician B.V. Petrovskiy, Moscow, Russia;

3Tambov Regional Children Clinical Hospital, Russia; 4Volgograd Regional Clinical Oncology Dispensary, Russia

Key words: Infl ammatory myofi broblastic tumor, children, rare tumor, ALK

Introduction. Infl ammatory myofi broblastic tumor (IMT) is a rare type of childhood malignancies with intermediate biologic behavior.

Aim. The aim of the study was to analyze clinical data and treatment results in a cohort of patients with IMT treated in four Russian children’s hospitals.

Materials and methods. 9 patients with IMT for the period of 01.2012–12.2015 (48 months) were included in the analyses. Central review of histology was obligatory. Anaplastic

lymphoma kinase (ALK) immunohistochemistry and FISH for ALK gene rearrangements were performed in all cases. Surgery was the fi rst treatment option.

Results. Male: female ratio was – 0.8:1. The median age at the diagnosis was 61.9 months (range 5.9–102.5). Tumor detection was an incidental fi nding in 3 (33.3 %) patients.

Most frequent symptom was fever – 4 (44.4 %). Tumor located in lungs – 4 (44.4 %), abdomen – 4 (44.4 %) and the liver – 1 (11.2 %). Regional lymph node involvement was observed

in 1 (11.1 %) case. Laboratory fi ndings included leukocytosis (4/9), anemia (5/9) and thrombocytosis (7/9). ALK expression was revealed in 4/9 (44.4 %) cases. ALK rearrangements

were not detected in 4 studied cases. All patients were operated. Extent of the surgery included gross total excision in 8/9 (88.9 %), biopsy – 1/9 (11.1 %). Microscopic positive margins

were confi rmed in 2/8 (25 %) cases. Surgery was the only therapy in 6/9 (66.7 %), 2/9 (22.2 %) patients with positive margins received celecoxib, 1/9 (11.1 %) with unresectable

tumor was treated with chemotherapy without response. All patients are alive. Median follow-up time was 10.5 months (range 1.3–27). 2/9 (22.2 %) relapses were observed.

Time to relapse was 5.9 and 9 months. Both patients received surgical procedure.

Conclusion. Our data confi rm lungs and abdomen as the most frequent sites of the disease. Surgery is the mainstay of therapy. More data are needed regarding the role of adjuvant

therapy in IMT.

A B S T R A C T N O . : P P - 2 4 3

Clinical experiences of infantile hepatic hemangioendothelioma

Jun Eun Park1, Leehuck Gil1, 2

1Ajou University School of Medicine Suwon, Korea; 2Ajou University Hospital, Suwon, Korea

Key words: hemangioendothelioma, infant, hepatic

Introduction. Infantile hepatic hemangioendothelioma is a rare but most common hepatic vascular neoplasm in less than six months old infants.

Aim. We tried to review the outcomes and treatment methods in infantile hepatic hemangioendothelioma.

Materials and methods. A retrospective review of 6 patients (2 males, 4 females) with infantile hepatic hemangioendothelioma at the Ajou University Hospital between 2001

and 2015 was performed.

Results. Six patients with infantile hepatic hemangioendothelioma were identifi ed. Median diagnosis with infantile hepatic hemangioendothelioma was 18 days (range,

1 to 91 days). Characteristics at the time of diagnosis were varied. Two patients were diagnosed with infantile hepatic hemangioendothelioma by prenatal ultrasonography,

2 had hepatomegaly, 1 had congestive heart failure, and 1 was diagnosed incidentally in the absence of symptoms. All patients were diagnosed by imaging study, and 2 patients were

confi rmed by pathologic diagnosis. Treatment options were selected by considering the patient’s symptoms and the characteristics of the tumor. Three patients were observed without

treatment, 1 patient with congestive heart failure and multilobar tumors was treated by hepatic artery embolization with medical therapy. One patient received interferon alpha

and subsequent surgical resection, 1 patient with possibility of hepatoblastoma received only surgical resection. Treatment outcome showed benign disease course of infantile hepatic

hemangioendothelioma. Overall, two patients without treatment revealed > 60 % decrease in tumor size and 1 patient without treatment showed complete tumor disappearance.

One patient who underwent surgical resection because of possibility of hepatoblastoma was in stable condition without recurrence. One patient who received interferon alpha during

6 months and subsequent surgical resection due to intermittent congestive heart failure symptoms showed > 50 % decrease in tumor size after medical treatment with complete

resolution of tumor after liver lobectomy. One patient who received initial hepatic artery embolization due to the unstable condition of congestive heart failure maintained 12 months

of steroid and 9 months of interferon alpha and showed > 50 % decrease in the size and number of lesions with degenerative change.

Conclusion. Infantile hepatic hemangioendothelioma is rare but can be cured with surgical or/and medical approaches. Clinical symptoms are key point in selecting treatment

options.

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A B S T R A C T N O . : P - 3 2 3

A case of neuroglial dystopia of nasopharynx, pterygomaxillary fossa, submandibular area

V. Alexeeva, L. Privalova, K. Isaev

Nizhny Novgorod Regional Children Clinical Hospital, Russia

Key words: neuroglial heterotopia, dystopia

Introduction. Heterotopy (dystopia, ectopia) is traditionally considered to be a congenital abnormality. During embryogenesis, due to the cells migrating from one primitive

tissue layer to another, the anlage place of an organ or tissue is changed, there is the replacement of cells within a germ layer, or secondary replacement of organs and/or tissues.

The tumours resulted from embryonic replacements are called heterotopic. Neuroglial heterotopias (NHs) are rare congenital abnormalities characterized by the presence

of diff erentiated tissues of neuroectodermal genesis in the places, where, as a rule, there are no neuron and glial cells. The places usually aff ected are head and neck areas,

and the lesions have been described more frequently in such extracranial loci as the nose, nasopharynx, oral cavity, oropharynx, palate, tongue, lips, scalp and orbits.

Aim. The purpose of the study was to demonstrate a case of neuroglial dystopia recorded in Nizhny Novgorod region.

Materials and methods. A 1-month-old girl, Arina K., was admitted to Nizhny Novgorod Regional Children Clinical Hospital on October, 23, 2015. The child was from the fi rst

pregnancy, the mother had a threatened miscarriage during the 7–8 weeks of gestation period. The delivery was fi rst, at term. The weight at birth was 3600.

Results. Since birth the newborn was noticed to have “snoring” breathing. The patient was found to have the right submandibular lesion, and 13 days after birth she was admitted to Nizhny

Novgorod Regional Children Clinical Hospital. On admission: full blood count dated 24.10.2015: Hb 165 g/l, leucocytes 15.4 × 109/l, platelets 523 × 109/l, ESR 1 mm/h. biochemical blood

assay: LDH 5.3 μkat/l-h, ALT 0.16, AST 0.31 (norm), urea 1.9 mmol/l, total bilirubin 41 μmol/l, total protein 56.6 g/l, electrolytes – norm, НСЕ 20 ng/ml, ferritin 227 ng/ml.

Brain neurosonography dated 24.10.2015 showed no signs of altered brain structures. Chest X-ray dated 24.10.2015 showed the mediastinum in the upper and middle respiratory

tract to be dilated to the right due to thymus, the trachea being tortuous.

Abdominal ultrasound dated 24.10.2015 revealed no pathology. Ultrasound of soft tissues dated 24.10.2015 showed the signs of the right cervical soft tissue lesion. Neck soft tissue

MRI (27.10.2015): the presentation of tumour of the cervical soft tissues on the right. On October, 28, 2015 the patient underwent the surgery for particle removal (biopsy) of the right

cervico-submandibular tumour. Histological and immunohistochemical studies revealed congenital neuroglial heterotopy. The patient was readmitted to the Oncology Department

for a follow-up. Cervical soft tissue ultrasound dated 15.12.2015: the signs of solid tumour on the right lateral neck side, 21.9 × 26 × 34.1 mm in size. Neurosonography dated

18.12.2015 revealed no pathology. Parotid MRI dated 18.12.2015 showed the signs of the lesion of the soft tissues of the right parotid area, irregularly shaped, with clear contours,

up to 23 × 37 × 37 mm in size, extending to the infra-temporal region and pterygomaxillary fossa, the nasopharynx soft tissues, pushing aside and deforming the right parotid

salivary gland. The laryngopharynx lumen is signifi cantly narrowed and deformed. Compared to the previous studies, no signifi cant changes were found.

Conclusion. Thus, the follow-up showed no negative changes. An oncologic follow-up is recommended and scheduled.

A B S T R A C T N O . : O P - 3 2 5

Rare types of lung tumors in children. Experience of Federal Research Center of Pediatric

Hematology, Oncology and Immunology named after Dmitriy Rogachev

D.Yu. Kachanov, M.V. Teleshova, T.V. Shamanskaya, A.M. Suleimanova, S.R. Talypov, N.N. Merkulov, R.S. Oganesyan,

N.G. Uskova, D.M. Konovalov, V.Yu. Roshchin, Yu.V. Olshanskaya, A.N. Kazakova, S.R. Varfolomeeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: infl ammatory myofi broblastic tumor, pleuropulmonary blastoma, fetal lung interstitial tumor, rare tumors, children

Introduction. Tumors of the lung in children are a heterogeneous group according to the degree of the tumor malignancy, extremely rare in a cohort of children up to 14 years.

Aim. To show and analyze the rare cases of primary lung tumors in children treated at the Federal scientifi c clinical center of Hematology, Oncology and Immunology in the period

of 12.2012–12.2015 years.

Materials and methods. in the FRCC in the period of 12.2012–12.2015 years (36 months) were treated 9 patients with primary malignant neoplasms of the lungs. We analyzed

the nosology, age at diagnosis, clinical and laboratory presentation of disease, stage. The diagnosis was established on the basis of histological examination in the laboratory of the

pathology FRCC. Reference of the part of histological samples were made in international registry of pleuropulmonary blastoma (USA). Patients received therapy according to the diagnoses.

Results. from 9 patients, 4 pts (44.4 %) had infl ammatory myofi broblastic tumor (IMT), 2 pts had pleuropulmonary blastoma (PPB), (22.2 %, 1pt – type I, transformation in type II;

1pt – type III), in 1 case (11.1 %) fetal lung interstitial tumor (FLIT) was confi rmed, 1pt (11.1 %) had mucoepidermal carcinoma (MEC) and 1pt (11.1 %) had leiomyosarcoma (LMS).

The median age at diagnosis was 51 months (4 months – 16 years). The patient with FLIT diagnosis was made in 4 months. Diagnosis of patient with LMS was verifi ed postmortem.

M/F racio was 1/1.25. In most cases, the disease was presented by initial pulmonary and intoxication symptoms (7 pts, 77.7 %), 2 pts (22.3 %) – the tumor was accidentally

detected during the examination. The tumor was localized in the right lung in 6 cases (66.6 %), in the left lung in 3 cases (33.4 %). Initial metastatic lesion was detected in 1 case

(12.5 %, PPB). All patients underwent surgical treatment: R0-resection – 5 cases (55.5 %, 4 pt. – IMT, 1pt. – MEC); R2-resection – 1 case (11.1 %, PPB), resection degree was unknown

in 2 cases (22.2 %, PPB, FLIT), autopsy was performed in 1 case (11.1 %, LMS). Chemotherapy in patients with PPB was performed according to the recommendations of the PPB registry.

In the case of a patient with FLIT chemotherapy was performed according to the recommendations for patients with PPB. After the revision of histological specimens in the PPB

registry, was recommended dynamic observation. IMT was subjected only to surgical interference. The median follow-up time was 19 months. (0.5 to 32 months). Outcome: 6 patients

alive, NED (66.6 %), 2 patients alive with relapse (22.2 %, PPB) alive with recurrence, 1 patient died before treatment begun of disease progression (11.2 %, IMT).

Conclusion. Malignant tumors with initial lung involvement in children are rare with a predominance of IMT. Outcome of the therapy directly depends on the of surgical intervention

radicality. The chemotherapy decision in patients with FLIT requires further discussion because of the extreme rarity of the disease and the short median follow-up.

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A B S T R A C T N O . : O P - 3 3 4

Intratissue infrared lazer thermotherapy

in the treatment of kaposiform hemangioendothelioma

I.A. Abushkin1, A.G. Denis2 1South Ural State Medical University, Chelyabinsk, Russia; 2Children’s Regional Hospital, Tver, Russia

Key words: intratissue infrared lazer thermotherapy, kaposiform hemangioendothelioma, minimally invasive method, diagnosis

Introduction. Kaposiform hemangioendothelioma (KHG) is a rare vascular tumor characterized by local aggressive growth. KHG is often accompanied by coagulopathy and thus,

is associated with Kasabach–Merritt phenomenon. KHG is typically registered in newborns and infants, in equal number of males and females. KHG in adults is extremely rare.

The prevalence of this disease is unknown. Published clinical trial data (S.E. Croteau et al., 2012) show that on average 0.071 cases of KHG per 100 000 children are diagnosed. A

cause factor or genetic predisposition to this type of tumors are unknown. The most common complication of the tumor is hemorrhage that in 10–40 % of cases results in death. For

the fi rst time this tumor was described as a self-consistent neoplasm in 1993 (L.R. Zukerberg et al.). Diagnosis: symptoms, complete blood count with platelets, fi ndings of Doppler

ultrasound (US), contrast magnetic resonance imaging/computed tomography. If the results of the above investigations are not informative, a histopathological examination is

performed. However, it should be taken into account the complexity of histopathology in the presence of concomitant thrombocytopenia and high risk of hemorrhage. There are many

methods of treatment: 1) surgical resection of tumor, that is not always possible owing to the risks stated above; 2) drug treatment with hormonotherapy, cytostatic drugs, Vincristine

and others, interferon-alpha; last year data on administration of the new medication Sirolimus were received; 3) X-ray treatment and embolization. Due to the fact that this disease

is rare, there is still no uniform adequate treatment approach.

Aim. The improvement of treatment outcomes of KHG.

Materials and methods. We used the method of intratissue infrared lazer thermotherapy in the treatment of KHG for the fi rst time ever. Semiconductor and fi ber-optic lasers

with the radiation wavelength of 970 and 1560 nm respectively were applied. Four children (an equal amount of boys and girls) aged 5–7 months old with various KHG localization

underwent treatment. KHG localization: in 1 child – in the region of superior surface of the lower leg (3 % of body surface area (BSA) involvement), in 1 child – in the lumbar region

(2 % of BSA), in 1 child – an extensive KHG in the thoracic region with the spread to the lateral surface of the trunk and to the back (7 % of BSA) and in 1 patient – an extensive KHG

in the abdominal region with the spread to the chest and lateral surface of the trunk (9 % of BSA). In children with extensive KHGs a concomitant Kasabach–Merritt phenomenon

was observed (platelets up to 20 × 109/l). For KHG treatment we applied the method of intratissue thermotherapy under ultrasound control with anaesthesia. Semiconductor laser

with the radiation wavelength of 970 nm and a fi ber-optic laser with the radiation wavelength of 1560 nm were used. All 4 patients were found to have false initial diagnosis:

lymphangioma, hemangioma, phlegmon of the trunk.

Results. In patients with extensive KHG, after the fi rst procedure of intratissue lazer thermotherapy tumors have decreased in size signifi cantly, platelet normalization was achieved.

Full recovery after 1 procedure was registered in the child with the disease localization in the lumbar region. Full recovery after 2 procedures was detected in the child with the disease

localization in the region of lower leg. After the 1st procedure of intratissue lazer thermotherapy, the signifi cant improvement with normalization of general condition was registered

in 2 children with extensive KHG.

Conclusion. Intratissue infrared lazer thermotherapy is a minimally invasive method of KHG treatment and may be recommended to wide use in cases of this rare and severe disorder.

A B S T R A C T N O . : P P - 3 8 2

Desmoplastic small round cell tumor in children and adolescents: single institution studyJung Yoon Choi, Hyoung Jin Lee, Che Ry Hong, Kyung Taek Hong, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin

Cancer Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea

Key words: desmoplastic small round cell tumor, pediatrics, Korea

Introduction. Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen

has not been established.

Aim. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients.

Materials and methods. We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National

University Children’s Hospital from January 1999 to January 2015.

Results. The median age at diagnosis was 11 years 5months (range 4 years 10 months – 17 years 2 months). The most frequent symptoms were abdominal pain (60 %) and palpable

mass (40 %). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had

multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after biopsy

confi rmation of the diagnosis. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5–54 Gy). Four patients showed

disease progression or relapse, resulting in a 20 % overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and was treated

with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.

Conclusion. Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.

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A B S T R A C T N O . : P P - 3 4 6

The nasopharyngeal cancer in children: Epidemiologic aspect and scalable

Sabah Sal

Residanat

Key words: chemosensitivity, radiocurabilite, nasopharyngeal, cancer, epithelial

Introduction. Malignant neoplasm of nasopharynx are mainly represented by the nasopharyngeal carcinoma (NPC) which is a tumor of epithelial origin. The most common

historical-clinical entity is undiff erentiated type nasopharyngeal carcinoma, the most common Carcinoma of the child who has a particular geographical distribution:

Algeria – MAGHREB, the relationship EBV-nasopharyngeal carcinoma is highlighted.

Aim. Through this work we studied the epidemiological, clinical and therapeutic characteristics in maghreb area.

Materials and methods. This work consists of a retrospective study of the epidemiological profi le of the nasopharyngeal carcinoma in childhood at the level of Department

of Medical Oncology Centre Pierre and Marie Curie, on a sample of 43 cases a period of 2009–2015.

Among the studied cases of nasopharyngeal cancer, 65 % were male and 32 % of female. We were 9 deaths due to such cancer among which 11.6 % were boys and 9.3 % of the girls.

At the level of the sample, the average age of children with nasopharyngeal carcinoma was 12 ± 3 years. Almost all children have received neoadjuvant chemotherapy and radio-

concomitant chemotherapy, the frequency of locally advanced stages was 25.58 % for girls and 37.2 % for boys.

Results. Rates of remission have been obtained; 23 % among girls and 55.8 % among boys, progressions well led post-treatment: 11.6 % among girls and 13.95 % among boys.

There were deaths: 9.3 % girls and 11.6 % boys.

Conclusion. The nasopharyngeal carcinoma is frequent in Algeria, it is lymphophilie and has a high metastatic potential.

It characterized by its radiocurabilite and its chemosensitivity, but the diagnostic delay exposes local and systemic dissemination.

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A B S T R A C T N O . : P - 3 8 4

Aggresive fi bromatosis in childhood at the oncologic pediatric service

of National Oncology Institute of Cuba (2003–2013)

Migdalia Perez, Mariuska Forteza, Jose Alert, Jesus Reno

National institute of Oncology and Radiobiology of Cuba, Havana

Key words: fi bromatosis, neoplasms

Introduction. Aggressive fi bromatosis, also called desmoide-tumor is a rare benign tumor, which originates from structures deep musculo-fascial and/or the periosteum;

and they are locally aggressive, prone to local recurrence. Not usually give distant metastases, but may infi ltrate vital structures, and be life enfermo. 1,2 comprising 0.03 % of

neoplasms in general and 3 % of all tumors parts blandas. 3,4 an incidence of 2–4 cases it is estimated per million inhabitants, and about 900 new cases are diagnosed annually in

United States. 5 Its etiology is unknown, has been associated with surgery and / or previous trauma, pregnancy, estrogen therapy and of Gardner syndrome.

The most common age of onset is during the fi rst decade of life, but there are reports of all ages. The most common locations are: abdominal. Histological diagnosis and treatment

is mainly the surgery.

Aim. To describe the clinical characteristics of and the treatment prescribed for patients with diagnosis of aggressive fi bromatosis, who were treated at the oncologic pediatrics service

of the National Institute of Oncology and Radiobiology.

Materials and methods. A descriptive, longitudinal and retrospective study. The universe consisted of all patients with malignant diseases in the INOR Oncopediatrics service;

and the sample consisted of 9 patients with histologically aggressive fi bromatosis, from 1 January 2003 to 31 December 2013, with prior approval of the informed consent of parents

or guardians in each case.

The variables studied were: age, sex, location, clinical presentation, imaging studies (plain radiographs and computed tomography (CT) and/or magnetic resonance imaging (MRI))

of the aff ected area as well as diagnostic biopsy immunohistochemical study all cases and treatment modality.

The analysis of contingency tables was used for the studied variables using Fisher’s exact test, with a confi dence interval of 95 %. The results were presented in tables and fi gures.

Data were processed in Excel and SPSS version 15.0.

Results. A total of 510 pediatric patients with malignant disease were evaluated in the selected period; of these, 9 had a diagnosis of aggressive fi bromatosis, with a slight

predominance of males (56 %) with a mean age of 9 years (range between 0 and 9), and only 22 % were older than 11 years.

The locations were: head and neck, accounting for 55 %, followed by gluteal and lower limbs. All patients underwent imaging studies. The diagnosis was made by incisional biopsy

of the lesion in all patients. The exclusive surgery was used only in 3 patients, and in other cases surgery radiotherapy (RTP) and chemotherapy (QTP) combined. Right now we have

a 100% survival.

Conclusion. The most common age of onset of aggressive fi bromatosis is under 10 years, with a slight predominance in males. The palpable tumor mass is the main clinical

manifestation, with a higher location in the head and neck. The main treatment is surgery, however, other therapeutic modalities should be included to achieve local control of the

disease.

A B S T R A C T N O . : P - 3 9 2

Results of investigation of main complex of HLA-phenotype at patients

with solid malignant tumoursYu.Yu. Kozel, S.A. Kuznetsov, M.V. Starzeckaya, G.A. Mkrtchyan

Rostov Research Oncology Institute, Rostov-on-Don, Russia

Key words: solid malignant tumours, main complex of histocompatibility

Introduction. Nowadays there is a theory that disease is a result of intercourse of environment and genetic factors. Among genetic factors the important place is taken by the main

complex of histocompatibility nowadays. There is a big interest now in investigation of correlation between tumours and HLA-specifi city. These investigations are important for early

diagnosing of oncological diseases.

Aim. Genetic typing of blood of children with solid malignant tumours for determination of prognosis of risk and forming of the risk groups.

Materials and methods. One hundred and thirty patients at age 1 to 18 years old treated in department of pediatric oncology of RROI with diff erent solid malignant tumours

were investigated: 21 (18.6 %) patients with neuroblastoma, 21 (18.6 %) with nephroblastoma, 20 (17.7 %) with osteosarcoma, 20 (17.7 %) with Ewing sarcoma, 31 (27.4 %)

with germinal tumours. Molecular typing of HLA-gene DRB1 performed with the help of polymerase-chain reaction, allowed to reveal 13 groups of alleles HLA-DRB. Statistical

diff erence was estimated with the help of χ2 criterion with Yates allowance for continuity for the small selection.

Results. Results of analysis of children with solid tumours in comparison with healthy donors revealed the signifi cant increasing of frequency of the following HLA-specifi cities:

А26 – 20 % (control – 5.35 %) (P < 0.02), B51 – 30 % (control – 12.35 %) (P < 0.05 %) – at Ewing sarcoma; А24 – 38.09 % (control – 16.46 %) (P < 0.02), B13 – 38.09 %

(control – 11.6 %) (P < 0.01), DRB 1*04 – 42.85 % (control – 18.75 %) (P < 0.05) – at neuroblastoma; DRB 1*13 – 57.14 % (control – 29.68 %) (P < 0.05) – at nephroblastoma;

А26 – 16.12 % (control – 5.35 %) (P < 0.05) – at germinal tumours.

Conclusion. Thus, in case of revealing the following alleles the child should be considered as a patient at a risk of Ewing sarcoma (А26, B51) (P < 0.05), neuroblastoma

(А24, В13, В44, DRB 1*04) (P < 0.01), nephroblastoma (DRB 1*13) (P < 0.05) and germinal tumours (А26) (P < 0.05).

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A B S T R A C T N O . : P P - 1 1 0

A case of hepatic veno-occlussive disease (VOD) presented as huge hemothorax after

the fi rst chemotherapy for childhood medulloblastoma

Chueh Hee Won, Lee Mi Ji, Joe Sun Young

Dong-A University Hospital

Key words: medulloblastoma, VOD, standard chemotherapy

Introduction. Hepatic veno-occlusive disease (VOD) is a serious toxic complication occurring after autologous and allogeneic hematopoietic stem cell transplant (HSCT). Also,

there were many reports on VOD associated with certain chemotherapeutic agents, such as actinomycin-D for the treatment of Wilms tumor and rhabdomyosarcoma, and 6-thioguanine

for acute lymphoblastic leukemia. However, VOD occurrence associated with regular chemotherapy is rare, and it has been regarded as “transplantation-related complication”.

Aim. We report a 21-months-old boy with high-risk medulloblastoma, who developed severe hepatic VOD presenting huge hemopthorax and ascites during his second cycle

of chemotherapy, and recovered after PGE1 treatment.

Materials and methods. Case report. 21-months-old boy was arrived to Dong-A University Hospital for the complaints of decreased mentality and seizure. Brain-imaging study

revealed large mass located in cerebellar area with severe hydrocephalus, and he received total resection of tumor operation. Biopsy confi rmed medulloblastoma, but he couldn’t

receive chemotherapy due to recurrent aggrevation of hydrocephalus. After receiving other two EVD insertion operation and V-P shunt operation, his mentality didn’t improved through

the hydrocephalus improved, and the MRI showed progression of the tumor with increased metastatic seeding through the ventricle and spine. Chemotherapy was started according

to the protocol of KSPNO-S1102, which was developed by Korean Society of Pediatric neurosurgery and oncology for the patients under 3 year with high-risk medulloblastoma.

His plan was receiving two diff erent chemotherapy regimens alternatively for 6 months, and receiving tandem high-dose chemotherapy with autologous stem cell transplantation.

Radiation therapy was planned to start after his age reach 3-year-old.

Results. He received chemotherapy with cisplatin, cyclophosphamide, etoposide and vincristine. On chemoday he showed high-grade fever with grade 5 neutropenia. When

the WBC counts recovered, he suddenly showed desaturation with haziness on both lung fi elds. Chest CT showed the large volume of eff usion – suspected hemorrhage. Chest tube

was inserted, and the large volume of blood got out continuously through the chest tube. He also showed large amount of ascites and bloody diarrhea. Blood test revealed that

he was in the state of hepatic VOD. FFP and albumin transfusion, continuous heparin and PGE1 infusion started. After 20 days later, VOD was completely recovered, and he could

undergo next cycle of chemotherapy. After that, the VOD didn’t relapse during the treatment course.

Conclusion. Our patients presented severe hepatic VOD with huge hemothorax after the single course of chemotherapy, which is not so intensive, and rarely considered to induce

VOD. There are a few reports of VOD occurred in standard chemotherapy setting, and all patients didn’t receive the chemotherapy agents which are known to be related to VOD. But

this is the second report of VOD occurred after the 1st chemotherapy in the patient with medulloblastoma. Therefore, VOD also should be considered as important complication during

the induction chemotherapy.

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A B S T R A C T N O . : O P - 1 1 4

Low-grade gliomas in children, associated with neurofi bromatosis type I R.R. Bayramgulov1, A.R. Bayramgulova1, A.A. Gumerov2, R.A. Gumerov1, V.B. Makhonin1, I.I. Badrtdinova1

1Republican Children’s Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: low-grade gliomas, astrocytoma, children, neurofi bromatosis, chemotherapy

Introduction. Low-grade gliomas (LGG) are the most common brain tumors in pediatric population comprising 30 % of primary central nervous system (CNS) tumors in children.

Neurofi bromatosis type I (NF-I) is a hereditary syndrome of which predisposition to the development of peripheral nervous system (PNS) and central nervous system (CNS) tumors

is typical. Low-grade gliomas (LGG) with optic nerve, chiasm and hypothalamus involvement develop in 10–12 % of children with NF-I.

Aim. To evaluate the results of treatment for low-grade gliomas (LGG) associated with NF-I in children who received treatment at our centre.

Materials and methods. 79 patients with low-grade gliomas (LGG), 8 of which (10.1 %) were associated with NF-I received treatment at SBHI RCCH (State Budgetary Healthcare

Institution “Republican Children’s Clinical Hospital”) from 12.05.2008 till 25.12.2015. All patients irrespective of age received chemotherapy (CT) with vincristine and carboplatin after

neurovisualization and surgery.

Results. Female patients prevailed (ratio 6:2). Distribution according to the age was the following: 1–4 years old – 3 (37.5 %) cases, 5–10 years – 2 (25 %) cases, older than 11 years

old – 3 (37.5 %) cases. Tumour localization in all patients is presented with visual pathways and hypothalamus. According to the Dodge classifi cation stage I was determined in 2 cases,

stage II was observed in 5 cases, stage III was determined in 1 case. Neurovisualization was performed in all patients after the manifestation of symptoms: ophthalmological in 8 cases,

neurologic – in 6 cases, general cerebral – in 3 cases, diencephalic syndrome – in 2 cases, endocrinological disorders – in 2 cases. In 1 (12.5 %) patient with chiasmatic-hypothalamic

tumor, diencephalic syndrome, endocrinopathy there was performed partial resection of diff use astrocytoma of WHO grade II followed with chemotherapy that was complicated

by lethal toxicity. Chemotherapy (CH) was performed after neurovisualization in 7 patients. Stabilization of the disease after chemotherapy was achieved in 6 cases, partial remission

was achieved in 1 case. 7-year overall survival and progression-free survival were 87.5 %.

Conclusion. The results of treatment for low-grade gliomas (LGG) associated with NF-I are favorable. The main indication to the administration of chemotherapy (CH) in case

of low-grade gliomas (LGG) and NF-I were ophthalmological symptoms, diencephalic syndrome. Factors of unfavorable prognosis in case of low-grade gliomas (LGG) associated with

NF-I are age above 5 years, chiasmatic-hypothalamic localization, diencephalic syndrome.

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Epidemiology of low-grade gliomas in children R.R. Bayramgulov1, A.R. Bayramgulova1, A.A. Gumerov2, R.A. Gumerov1, V.B. Makhonin1, I.I. Badrtdinova1

1Republican Children’s Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: low-grade gliomas, astrocytoma, children, epidemiology

Introduction. Low-grade gliomas (LGG) represent a heterogenous group of central nervous system (CNS) tumours in children that comprises 30–50 %. There is a suggestion that

the number of these tumours is understated in children’s cancer registers that is partially due to the limited referral of patients to specialized centres.

Aim. To investigate epidemiological peculiarities of low-grade gliomas (LGG) in children by the experience of our centre.

Materials and methods. We have carried out the analysis of the patients who received treatment for low-grade gliomas (LGG) at the SBHI RCCH (State Budgetary Healthcare

Institution “Republican Children’s Clinical Hospital”) during 12.05.2008 – 25.12.2015. Data about the patients was obtained from medical documents with identifi cation

of patients with the histologically proved diagnosis of low-grade gliomas (LGG). Data analysis included age, sex, association with neurofi bromatosis type I (NF-I). Moreover data

of neurovisualization was analyzed in order to specify the tumour localization and defi ne involved brain structures.

Results. During the stated period 79 children with low-grade gliomas (LGG) remained under observation at SBHI RCCH. 49 male patients, 30 female patients (ratio was 1.6:2)

were registered. 8 (10.1 %) of 79 patients with low-grade gliomas (LGG) were associated with NF-I. Age distribution among the patients with NF-I was the following: 1–4 years

old – 3 (37.5 %) cases, 5–10 years old – 2 (25 %) cases, older that 11 years old – 3 (37.5 %) cases. Brain hemispheres were involved in 10 (12.2 %) cases, of which the tumour

localization in temporal lobe was in 6 children, in frontal lobe in 2 children, in occipital lobe in 1 child, there was 1 case with the involvement of several lobes.

Supratentorial medial localization was presented in 30 (36.6 %) patients, optic nerves were involved in 2 cases of them, chiasm was involved in 6 cases of them, optic nerves/chiasm –

in 3 cases of them, optic nerves/chiasm/ hypothalamus – in 4 cases of them, chiasm/ thalamus/ hypothalamus – in 3 cases of them, chiasm/ III ventricle – 1 observation, chiasm/ III

ventricle/ hypothalamus – 2, thalamus – 5, thalamus/ III ventricle – 1, other medial structures – 3 observations. The involvement of cerebellum was observed in 30 (36.6 %) cases,

of them the involvement of the right hemisphere was noted in 5 observations, the left hemisphere – in 4, vermis – in 10 cases, the right hemisphere/vermis – in 4, the left hemisphere/

vermis – in 3, both hemispheres/vermis – in 4. Brainstem was involved in 3 children, spinal cord – in 2 (2.4 %) at the level of С2-Th9, C1-Th10. Other brain structures were involved

in 10 (12.2 %) patients. 3 children had several tumours of diff erent localizations. Tumour localization in all patients with NF-I was presented with visual pathways, hypothalamus.

Histology is verifi ed in 71 patients: pilocytic astrocytoma (Ac) I – 30 cases, desmoplastic infantile ganglioglioma I – 1, pleomorphic xanthoastrocytoma II – 1, diff use astrocytoma (Ac)

II – 27, other – 10. In 8 cases with low-grade gliomas (LGG), associated with NF-I verifi cation was not necessary.

Conclusion. The predominance of male patients was observed. Typically low-grade gliomas (LGG) are diagnosed at the age of 1–10 years old. The association with NF-I is noted

in 10.1 %. Main localizations of low-grade gliomas (LGG) are medial supratentorial and cerebellum. In most cases tumour histology was presented with pilocytic astrocytoma (Ac)

and diff use astrocytomas (Ac).

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Placement of the Ommaya reservoir in narrow and slit-like ventricles

using a neuronavigation system

S.S. Ozerov1, A.E. Samarin1, A.V. Melnikov2, E.V. Kumirova1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;2Research Institute of Emergency Children’s Surgery and Traumatology, Moscow, Russia

Key words: Ommaya reservoir, intraventricular chemotherapy, methotrexate, medulloblastoma

Introduction. Recently, a remarkable progress has been achieved in the treatment of patients with malignant tumors of the central nervous system (CNS) and oncohematological

diseases. An important part of treatment protocols is intraventricular administration of methotrexate and other chemotherapy agents. An essential requirement for the conduction

of intraventricular chemotherapy is availability of an Ommaya reservoir, a special port that enables physicians to perform multiple and painless sampling of ventricular liquor

and deliver chemotherapy agents into the ventricular system of the brain. In most cases the placement of Ommaya reservoir is obstructed by the small sizes of the ventricles

of the brain. Nowadays, the insertion of Ommaya reservoir in patients with narrow ventricles of the brain is performed either with the use of stereotactic frame or neuronavigation.

In order to simplify and reduce the price of catheterization of narrow ventricles of the brain for the placement of an Ommaya reservoir and shunt systems, the method of stereotactic

placement of Ommaya reservoir and catheterization of narrow ventricles of the brain with the use of special ventricular guide (Thomale-guide) was studied and implemented in our

Centre for the fi rst time in Russia. This method make it possible to insert Ommaya reservoirs and ventricular catheters into narrow ventricles of the brain without the use of stereotactic

frame and expensive navigation systems.

Aim. To simplify and optimize the method of Ommaya reservoir placement, to achieve a high precision of reservoir placement in patients with narrow and slit-like ventricles without

the use of expensive navigation and stereotactic systems.

Materials and methods. An Ommaya reservoir is a convenient and safe port for intraventricular delivery of chemotherapy agents in patients with oncohematological

and neurooncological diseases. From 2012 until 2016 at the FRC PHOI n.a. Dmitry Rogachev 56 Ommaya reservoirs were inserted in 56 patients.

The reservoir was inserted without the use of any devices in 3 cases. However, in most cases the procedure was performed with the help of stereotactic techniques owing to small

sizes of the ventricular system of the brain. The reservoir was inserted by means of neuronavigator in 47 patients. In 6 patients stereotactic procedure was carried out based on a new

technology with the use of Thomale-guide, that makes it possible to simplify and accelerate the intervention while maintaining necessary precision.

Results. In all the cases a catheter was inserted into the anterior horn of lateral ventricle on the fi rst attempt, that was confi rmed by successful functioning of Ommaya reservoir

as well as by the results of postoperative computed tomography or magnetic resonance imaging. In 1 patient the placement of Ommaya reservoir was performed along with

a stereotactic biopsy. We did not observe any complications described in the literature such as infectious complications, hemorrhage, improper placement of a ventricular catheter.

In 2 patients a few months after operation we registered the dysfunction of the reservoir caused by the displacement of a pump in the subcutaneous pocket that resulted in constriction

of a ventricular catheter. In both cases normal functioning of the system was restored after the revision of the pump.

Conclusion. An Ommaya reservoir is a convenient and safe port for intraventricular delivery of chemotherapy agents in patients with oncohematological and neurooncological

diseases. A Thomale-guide enables us to achieve high precision during the insertion of Ommaya reservoir and can substitute navigation stereotactic systems.

A B S T R A C T N O . : O P - 1 4 8

The role of adjuvant chemotherapy for radiation therapy of diff usely growing tumors

of the brain stem in children

O.I. Shcherbenko, R. Parhomenko, N. Zelinskaya, F. Antonenko

Russian Scientifi c Center of Roentgenology and Radiology, Moscow, Russia

Key words: children, brain stem tumors, radiation therapy, chemotherapy

Introduction. The poor results of radiation therapy of diff use brain stem tumors are forced to combine the irradiation with the use of chemotherapy

Aim. To evaluate the contribution of chemotherapy to the radiation treatment results

Materials and methods. We carried out the analysis of immediate and long-term results of radiotherapy or chemoradiotherapy in 120 children and adolescents with inoperable

diff usely growing tumors of the brain stem, treated in the Children’s Department of RSCRR in 1998–2013. Age of patients ranged from 2 to 18 years, median age was 8 years.

In 22 patients biopsy of the tumor was performed: 8 cases were glioblastomas, 5 patients had pilocytic astrocytomas, 4 cases were anaplastic astrocytomas, 4 – fi brillary astrocytomas

and one patient had a diff use astrocytoma. In 93 patients radiotherapy was combined with adjuvant chemotherapy: Temozolomide (41), the combination of Vincristine and Lomustine

(19), Ftorafur (11), Oncofer (16), Teralok (6). 27 children received only radiation therapy.

Results. Treatment was completed in 117 patients, the treatment of three patients was interrupted due to worsening of their condition. The main toxic eff ects in the form

the myelodepression and neuropathy was observed in some patients receiving chemotherapy. 110 of 117 patients, who underwent the whole course of irradiation (94 %), achieved

complete or partial regression of neurological disorders. Upon further observation 113 children died. In all cases the cause of death was tumor progression. 7 patients have been

observed for the period from 29 months to 152 months. Average life expectancy for the entire group was 13.5 ± 11.3 month: 13.7 ± 10.2 months after chemoradiation treatment

and 13.2 ± 11.1 months after only radiation therapy. Six of ten 2-year survivors had morphological confi rmation: 4 cases had pilocytic astrocytoma, one had fi brillar astrocytoma

and one had diff use astrocytoma. All glioblastoma patients died within the fi rst 6 months.

Conclusion. The prognosis in the diff use growing brain stem tumors completely depends on the degree of malignancy. Adjuvant chemotherapy in several variants used by us did not

improve the results of radiation therapy, but led to an increase of economic costs and in some patients to the development of toxic complications.

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A B S T R A C T N O . : P P - 1 6 8

The responsiveness of pediatric brain tumor patients with poor prognostic factors

to combination treatment with oral temozolomide and radiotherapy

Tamami Yano, Michihiro Yano, Miwa Hebiguchi, Koya Kodama, Tsutomu Takahashi

Akita University Hospital

Key words: recurrent brain tumor, temozolomide, radiotherapy

Introduction. Some trials are being performed with the aim of improving the treatment outcomes of pediatric patients with brain tumors and poor prognostic factors. Temozolomide

(TMZ) is a candidate drug that is expected to aff ect these tumors.

Aim. We observed the clinical course and results of patients with unresectable pontine gliomas or recurrent brain tumors who received combination treatment with oral TMZ

and radiotherapy.

Materials and methods. TMZ was administered orally with radiotherapy to the pontine glioma patients. The patients with other recurrent brain tumors were fi rst managed

in the same manner as the patients with pontine gliomas; TMZ then was administered alone.

Results. Case 1. A six-year-old female with a pontine glioma. TMZ was administered with local radiotherapy (54 Gy). She died of the disease at 8 months after her diagnosis.

Glioblastoma was positively identifi ed at autopsy. Case 2. A nine-year-old male with a pontine glioma. TMZ was administered with local radiotherapy (56 Gy). The patient died of the

disease at 21 months after his diagnosis. Case 3. A four-month-old female who was initially diagnosed with a glioblastoma of the left parietal lobe. The patient’s tumor was persistent,

despite the administration of two courses of combined chemotherapy after tumor resection. TMZ and radiotherapy were administered, followed by TMZ alone for approximately

3 years. Five years after the termination of TMZ, she has shown no signs of disease reprogression. Case 4. A one-year-old male who was initially diagnosed with a medulloblastoma

of the fourth ventricle and disseminated spinal metastases. According to the study protocol of a Japanese group, he underwent six courses of combined chemotherapy, several courses

of intrathecal chemotherapies, and two courses of high-dose chemotherapy with autologous bone marrow transplantation. Multiple relapsed nodules were found in his cerebral

ventricle and on the surface of his spine at one year after treatment. TMZ and radiotherapy were administered, followed by TMZ alone for approximately 6 months. Five and a half

years after the termination of TMZ, he has shown no signs of disease reprogression.

Conclusion. In the cases of pontine glioma (cases 1 and 2), the prognosis was considered to be poor because tumor resection was impossible. Bevacizumab, which is an anti-

vascular endothelial growth factor (VEGF) monoclonal antibody, is expected to improve the prognosis of pontine glioma. The treatment outcomes of cases 3 and 4 show the potential

of TMZ in improving the prognosis of patients with recurrent pediatric brain tumors. However, the contribution of radiotherapy should not be ignored. Furthermore, physicians should

be vigilant for secondary malignancies, which may be induced by the long-term administration of TMZ and careful care should be taken in the long-term follow up.

A B S T R A C T N O . : P P - 1 6 9

An eff ective stereotactic radiotherapy for recurrent pilomyxoid astrocytoma

in the medulla oblongata of pediatric patient

Tamami Yano, Michihiro Yano, Miwa Hebiguchi, Koya Kodama, Tsutomu Takahashi

Akita University Hospital

Key words: pilomyxoid astrocytoma, medulla oblongata, recurrent tumor, combination chemotherapy, stereotactic radiotherapy

Introduction. Pilomyxoid astrocytoma (PMA), which mainly arises within the hypothalamic-chiasmatic region, was classifi ed as a WHO grade II disease in 2007. PMA is associated

with a poor prognosis in comparison to pilocytic astrocytoma (PA, WHO grade I). It is necessary to search for a recommended treatment strategy for PMA.

Aim. Stereotactic radiotherapy (SRT) was administered to a pediatric patient with recurrent PMA in the medulla oblongata.

Materials and methods. Case. A ten-month-old female presented with hoarseness, torticollis, diffi culty of swallowing and poor body weight gain. MRI revealed

that a heterogeneously enhanced mass projected from her medulla oblongata toward the left cerebellar hemispheres. The tumor was removed to the maximum possible extent.

A histopathological examination revealed characteristics of PMA. Following surgery, four courses of combination chemotherapy consisting of cisplatin, etoposide, cyclophosphamide

and vincristine were administered, and her neurological symptoms gradually disappeared. About two years after the completion of chemotherapy, the remaining tumor, that had

initially been unresectable, began to slowly grow in size without any symptoms.

Results. We administered SRT (36 Gy, 9 Fr) because chemotherapy was not expected to have further eff ects. None of side eff ects of SRT occurred. Now, 4 years after SRT, the tumor is

gradually becoming smaller and the patient is able to attend elementary school.

Conclusion. At present, the treatment strategies for PMA remain controversial, and are more controversial in patients with recurrent disease. The eff ectiveness of radiotherapy

remains to be elucidated in both PA and PMA. As seen in this PMA case, SRT is an eff ective management strategy when a relapse occurs in spite of a good response to the initial

chemotherapy.

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A B S T R A C T N O . : O P - 1 7 0

Surgical treatment of low-grade gliomas (LGG) in children

R.R. Bayramgulov1, A.R. Bayramgulova1, A.A. Gumerov2, R.A. Gumerov1, I.I. Badrtdinova1, V.B. Makhonin1

1Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: low-grade gliomas (LGG), astrocytoma, children, surgery, extent of resection

Introduction. Low-grade gliomas (LGG) are the most common brain tumors in pediatric population comprising 30 % of primary central nervous system tumours in children.

The main treatment method is surgery but it is not always feasible due to anatomical localization of the tumour that in some cases requires non-surgical treatment.

Aim. To evaluate the results of surgical treatment for LGG in children who received treatment at our centre.

Materials and methods. There was carried out the analysis of medical documents of the patients with LGG who received treatment at our centre during the period from 12.05.2008

till 25.12.2015. Neurovisualization prior to the surgery defi ned tumour localization, involved structures, tumour volume. Neurovisualization was performed after the surgery within

24–72 hours in order to evaluate the extent of resection. Moreover, the analysis of the response to the treatment, overall survival and progression-free survival were carried out.

Results. During the stated period 79 patients with LGG were enrolled, tumour resection was performed in case of 72 (91.1 %) of them. 47 (65.3 %) patients were male, 25 (34.7 %)

patients were female. Age distribution: < 1 year old (n = 8), 1–4 years old (n = 23), 5–10 years old (n = 22), older than 11 years old (n = 9). After surgery 55 (76.4 %) children were

referred to the observation group, 14 (19.4 %) patients required non-surgical treatment (chemotherapy (n = 8), radiotherapy (n = 7), both methods (n = 1)), there was a refusal of

treatment in 3 cases. Tumour localization: brain hemispheres (n = 10), supratentorial medial structures (n = 23), cerebellum (n = 30), spinal cord (n = 2), other structures (n = 10).

Tumour volume prior to an operation was evaluated in 55 children: less than 50 cm3 (n = 35), 50–100 сm3 (n = 12), more than 100 cm3 (n = 8). The extent of resection: total resection

31.9 % (n = 23), subtotal resection 25 % (n = 18), partial resection 33.4 % (n = 24), biopsy 9.7 % (n = 7). Histology was verifi ed in 71 patients: pilocytic astrocytoma (Ас) I – 30 cases

(42.2 %), desmoplastic infantile ganglioglioma I – 1 (2.8 %), pleomorphic xanthoastrocytoma II – 1, diff use Ac II – 27 (38.1 %), others – 10 (14.1 %). In 8 cases of low grade gliomas

(LGG) associated with NF-1 verifi cation was not required. The disease progression was observed in 3 children (4.2 %), the disease stabilization was registered in 9 (12.5 %) cases,

partial response – 18 (25 %) cases, complete response – 37 (51.4 %) observations. 5 (6.9 %) children died: of complications from surgery (n = 3), of complications from chemotherapy

(n = 1), of disease progression (n = 1). Overall survival comprised 93 %, progression-free survival – 90.3 %.

Conclusion. Surgery remains the basis of therapy for LGG in children. Total and subtotal resection are the most consistent prognostic factor defi ning overall survival and progression-

free survival.

A B S T R A C T N O . : P - 1 8 4

Reirradiation treatment of diff use brain stem tumors in children

O.I. Shcherbenko, O. Geludkova, R. Parhomenko, N. Zelinskaya, F. Antonenko

Russian Scientifi c Center of Roentgenology and Radiology, Moscow, Russia

Key words: Tumors of the brain stem, repeated radiation therapy, children.

Introduction. A local relapse of tumour of brainstem is principal reason of failures after conservative treatment. In practice such patients usually fall into a category incurable

and undergo only to symptomatic therapy.

Aim. To evaluate the possibility and eff ectiveness of the reirradiation treatment of diff use brain stem tumors in children.

Materials and methods. For 2001–2011 repeated radiation treatment was performed in 20 children with diff use brain stem tumors. All the children had previously received

radiation therapy with irradiation of the tumor in a total dose of 50–55 Gy and 7 in combine with chemotherapy by temozolomide . The reason for conducting the re-treatment

was the resumed tumor growth, proven by clinical and radiological data. The interval between the end of the initial treatment and the beginning of reirradiation ranged from

5 to 32 months, the median interval was 12 months. Re-radiation therapy was combined with adjuvant chemotherapy: temozolomide in 10 patients and bevazisumab in three.

Total dose of re-irradiation was less than 30 Gy in 10 patients, 31-45 Gy in 9 and 50 Gy in 1.

Results. In the process of treatment the condition of the 5 patients, who had radiological signs of the destruction of the tumor, worsened, and therapy was discontinued. Their median

survival was 3.5 months. In all others, who had no MRI signs of the tumor destruction, a positive subjective eff ects were noted in the form of full or partial regression of neurological

disorders. In this group 93 % survived 6 months from the beginning of the second course, 53 % survived one year, 40 % – 1.5 years, 20 % – 2 years. One patient survived over fi ve-year

period, he has lived for 13 years. In one case, 5 months after the re-irradiation at a dose of 50 Gy, symmetric foci of necrosis in the cerebellar hemispheres were identifi ed, combined

with persistent tumor growth.

Conclusion. Repeated radiation therapy for the regrowth of diff use brain stem tumors without signs of destruction can improve quality of life and increase survival in most cases.

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A B S T R A C T N O . : P P - 1 8 6

High-dose chemotherapy with autologous bone marrow transplantation

in the treatment of pediatric brain tumors

A. Gevorgian1, E. Morozova1, I. Kazantsev1, T. Iukhta1, S. Safonova1, P. Tolkunova1, Yu. Punanov1,

L. Zubarovskaya1, O. Zheludkova2, B. Afanasyev1

1First Pavlov State Medical University of St.Petersburg, Saint Petersburg, Russia; 2Russian Scientifi c Center of Roentgenology and Radiology, Moscow, Russia

Key words: pediatric brain tumors, high-dose chemotherapy, autologous hematopoietic stem cell transplantation, medulloblastoma, PNET, germ cell tumors, ATRT

Introduction. Central nervous system (CNS) tumors are the second most common pediatric malignancies with an about 30 % 5-year overall survival rate in high-risk group.

Aim. The aim of this study was to assess the eff ectiveness of high-dose chemotherapy (HDCT) with autologous hematopoietic stem-cell transplantation (auto-HSCT) in this patient group.

Materials and methods. From 2008 to 2015, 54 pediatric patients with high-risk or relapsed medulloblastoma (n = 32), supratentorial PNET (n = 8), germinoma

(n = 6), pineoblastoma (n = 3), atypical teratoid rhabdoid tumor (n = 3), choriocarcinoma (n = 1), ETANTR (n = 1) received single or tandem HDCT with auto-HSCT after induction

chemotherapy, radiotherapy and surgical treatment. At the moment of HDCT 25 patients were in complete remission (CR), 24 patients were in partial remission (PR) and 5 patients had

stable disease (SD). The conditioning regimen for single auto-HDCT (n = 47) consisted of cisplatin, etoposide, and ifosfamide, or carboplatin, etoposide and thiotepa ± intraventricular

etoposide, or thiotepa and temozolomide. In tandem HDCT (n = 7), the fi rst conditioning regimen was carboplatin and etoposide, the second was thiotepa and cyclophosphamide,

both with intraventricular/intrathecal metotrexat.

Results. The median follow-up is 48 months (range, 5–173). The median time to engraftment was day +17 (range, 8–86) after auto-HSCT. Four of 5 patients with SD at the moment

of auto-HSCT had disease progression within 8 months after HDCT. Twenty-two of 49 patients with CR or PR relapsed 1–24 months after HDCT, the other 27 patients are currently

in CR or PR on the maintenance therapy. Cumulative incidence of relapse in 4 years accounted 45 % (95 % CI: 21–60 %). The conditioning regimens had acceptable toxicity.

Complications grade 4 (COMMON TOXICITY CRITERIA 2014) were observed in 14 % of cases. Four-year overall survival (OS) in all patient’s group was 67 % and disease free survival (DFS)

was 55 %. MB and germ cell tumors had better survival rate (DFS 65 % and 64 %, respectively) in compared to other embrional tumors (DFS 40 %, P = 0.05). DFS was signifi cantly

better among patients > 4year in compared to children.

Conclusion. HDCT with auto-HSCT in pediatric patients with high-risk CNS tumors may be a feasible option for patients in CR or PR after induction chemotherapy. It is ineff ective as

a salvage therapy in refractory patients.

A B S T R A C T N O . : P - 1 9 1

Epidemiological characteristics of cerebral tumours in children

in Nizhny Novgorod region

L. Karaseva1, L. Privalova1, O. Zheludkova2

1Nizhny Novgorod Regional Children Clinical Hospital, Russia; 2Russian Scientifi c Center of Roentgenology and Radiology, Moscow, Russia

Key words: epidemiological characteristics cerebral tumours

Introduction. Central nervous system tumours steadily rank next to haematological malignancies among children in Nizhny Novgorod region. During the period from 2006 to 2010

there have been recorded 5–7 primary cerebral tumours, while within the period from 2011 to 2014, 12 tumours have been diagnosed. The highest incidence rate usually fell within

the autumn months (October, November). Currently, the number of patients with primary disseminated tumour forms is progressively increasing that has signifi cantly complicated

the capabilities of complex therapy and infl uenced the prognosis.

Aim. The aim of the investigation was to study the epidemiological characteristics of central nervous system malignancies in children in Nizhny Novgorod region.

Materials and methods. During the last 9 years (2006–2014), 77 patients with brain tumours were receiving complex therapy in the Oncology Department of Nizhny Novgorod

Regional Children Clinical Hospital.

Results. Among them, 38 patients were the citizens of Nizhny Novgorod and Dzerzhinsk: large industrial centres with urban highway networks, and packed with automobile,

chemical plants and oil refi neries. The analysis of diseased children in Nizhny Novgorod showed that more than half of the patients were from Zarechnaya part of the city

(Avtozavodsky, Sormovsky, Moskovsky districts), where township-forming enterprises are situated, the children population density being the highest. Three patients were under

3 years at the moment when the diagnosis was made. In 60 % cases, at birth, the mothers of the children under study aged over 30. Some mothers had burdened gynaecological

and obstetric history. Five women had had previous missed miscarriages before the birth of children with CNS tumours. Five women had suff ered from recurrent miscarriages,

and fi ve mothers had been receiving the therapy for infertility of mixed genesis for 2 years. One child was born after ICSI (intracytoplasmic sperm injection) procedure. The mothers

of all the patients (77) were found to have had severe gestational toxicosis of the fi rst trimester of pregnancy. Two mothers had previous dead births. In 90 % cases the mothers had

bad habits (smoking), and they had kept smoking during pregnancy. The study of the anatomical structure of CNS tumours showed the growing number of patients with primary

lesions of the brain stem: 13 patients (16.8 %).

Conclusion. Thus, environmental conditions have a signifi cant eff ect on CNS cancer morbidity rate in children in Nizhny Novgorod region. Burdened gynaecological and obstetric

history, age, and bad habits of mothers have a signifi cant impact on the increase in the number of patients with brain tumours. Fertility specialists should be concerned with the fact

and show oncologic suspicion under the present situation. The causes of an increased number of patients with brain stem tumours require further investigations.

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Neurocognitive disorders in children with post cranial fossa tumour

I.D. Borodina, R.B. Miroshkin, E.V. Fisun, A.S. Pchelinceva

Treatment and Rehabilitation Scientifi c Centre “Russkoe pole” of Federal Research Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: brain tumors, neurocognitive disorders, children

Introduction. Neurocognitive disorders are among the most common treatment complications in patients with brain tumors which do not receive due consideration owing

to the diffi culties connected with their identifi cation and assessment during the standard consultation of oncologist, neurologist or psychologist. At present, the test battery CANTAB

(Cambridge Neuropsychologic Test Automated Battery) designed to provide solutions of this problem meets international requirements optimally.

Aim. To estimate neurocognitive disorders in patients with post cranial fossa tumours.

Materials and methods. In the framework of the study of neurocognitive disorders in children with post cranial fossa tumours with the CANTAB we have examined 26 patients aged

6–17 years old (median age at examination – 11.5 years old), among them were 14 boys (53.83 %) and 12 girls (46.15 %). Twenty-three patients (88.47 %) had medulloblastoma

(MB) diagnosis, 2 (7.69 %) patients had pilocytic astrocytoma (PA), 1 (3.84 %) patient – anaplastic ependymoma (AE). All the patients (100.00 %) had underwent surgical treatment.

After surgery, twenty-four patients with malignant neoplasms (MB and AE) received radiochemotherapy or/and polychemotherapy according to the HIT 2000–2008 protocol

depending on their hystological diagnosis, age and the extent of tumour process (7 patients with stage М0 tumours, 3 patients with stage М1 tumours, 8 patients with stage

М3 tumours, 6 patients with stage Мх). One of 2 patients with benign tumours (PA) received surgery alone, the other one underwent surgery and radiotherapy. Median age

at the beginning of therapy was 6.5 (3–16) years old. After the end of antitumour therapy, 5 (19.23 %) patients had tumour progression (relapses, metastases and continued

tumour growth) and underwent repeated surgical treatment, radiochemotherapy and/or anti-relapse chemotherapy. By the time of examination, all the patients have completed

the treatment of main disease: 23 (88.47 %) patients demonstrated complete response, 3 (11.53 %) patients showed disease stabilization without treatment (median time after

the completion of treatment and before the start of the CANTAB testing is 15 (1–102) months.

Results. The study of cognitive functions in children with post cranial fossa tumours as compared to normal ranges for healthy child population revealed the following details: 100 %

of patients have visual, motor and conceptual diffi culties of various degree; 82 % of the examined children demonstrate the degradation of intelligence, educability and refocusing;

63 % of children were found to have a decrease in visuospatial memory span; 33 % of patients did not manage spatial tasks and motor control, 48 % of subjects experienced diffi culties

in the solution of this kind of tasks; 12 % of children demonstrate signs of impulsivity, 21 % – signs of retardation.

Conclusion. Neurocognitive disorders are detected in all patients with post cranial fossa tumours despite tumour grade, extent of treatment, age and timing of treatment.

A B S T R A C T N O . : P P - 2 1 0

Brain tumors infant, experience of pediatric oncology unit in Algiers

Cherifa Louni1, Fatiha Gachi1, Souad Bakhti2, Fella Terkmani2

1Centre Pierre & Marie Curie, Algeria; 2CHU Mustapha, Alger, Algeria

Key words: infant, brain tumors, chemotherapy, radiotherapy

Introduction. 10–20 % of childhood brain tumors occur before the age of 3 years. Treatment is often aggressive consisting of surgery followed by radiation source neurological

damage, endocrine and neuropsychological . The goal of chemotherapy is to delay or avoid irradiation in these young children.

Materials and methods. A retrospective study by analyzing records on 50 children aged under 36 months bearers of brain tumors treated between January 2008

and December 2014. The diagnosis was made by histology after surgery or stereotactic biopsy or imaging (inaccessible tumors). The treatment consisted of fi rst surgery unless

the anatomical exposes to unacceptable eff ects of chemotherapy followed by SFOP BABY protocol.

Results. 30 % of patients were younger than 18 months, with a male predominance (sex ratio: 0.56). The clinical picture is dominated by a intracranian hypertension syndrome.

A imaging, large tumors and surgery was often incomplete. According to the histological type (medulloblastoma 40 %, 18 % high-grade astrocytoma, 16% low grade gliomas,

épendymome 14 % and 12 % neuroectodermal tumors). All received chemotherapy according to SFOP BEBE protocol. 3 children were irradiated at the age of 03 years (ependymoma).

29 children died. The 21 survivors were not irradiated but have neurologic sequel of surgery.

Conclusion. Diagnosis is often delayed at the stage or tumors are very large making it diffi cult and incomplete surgery. The prognosis of brain tumors in children under three years

is worse compared to older. Conventional chemotherapy is not always eff ective, the use of intensive chemotherapy, conformal radiotherapy and the development of specifi c protocols

each tumor will improve outcomes for these children.

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A B S T R A C T N O . : O P - 2 7 6

Review on the management & outcome of childhood cerebral

or cerebellar low grade glioma

Godfrey Chi-Fung Chan1, Matthew Ming-Kong Shing2, Kai-On Chang3, Dennis Tak-Noi Ku4,

Alvin Siu-Cheung Ling5, Anthony Pak-Yin Liu1

1The University of Hong Kong; 2Prince of Wales Hospital; 3Queen Elizabeth Hospital;4Tuen Mun Hospital; 5Princess Margaret Hospital

Key words: low grade glioma

Introduction. Astrocytoma is the commonest type of brain tumors in childhood and low grade (WHO Gr I & II) accounted for almost half of them. Despite having good prognosis, the

management approach remains quite variable in diff erent parts of the world.

Aim. We reviewed our experience on this group of patients.

Materials and methods. This is a retrospective review of a population based cohort from Jan-1999 to Dec-2014. The patients underwent treatment in 5 local hospitals which

captured almost all children with cancers locally. The patients’ data were recorded prospectively by 2 full time data managers. The data was verifi ed annually with the respective

centers and HK Cancer registry.

Results. Within this 16 yrs period, there were 157 children with astrocytoma diagnosed. They were distributed as following: Cerebral & Cerebellar: n = 107 (Gr I n = 44, Gr II n = 21,

Gr III n = 14, Gr IV n = 28); Brainstem: n = 50; Spinal: n = 9 (Gr I n = 4, Gr II n = 1, Gr III n = 1, Gr IV n = 4); Other types of glioma: n = 20 (Oligodendroglioma n = 10,

PXA n = 2, Ganglioglioma n = 7, SEGA n = 1). Excluding the spinal astrocytoma and other types of glioma, we identifi ed 65 patients with cerebral or cerebellar low grade astrocytoma

as mentioned above. Their median age was 7.95yrs (range 0.15 to 17.81yrs) and M:F = 35:30. Cerebellum is the most common location (n = 28) followed by suprasellar region (n = 10).

Surgery was the main form of treatment (n = 57) and 18 received additional low intensity chemotherapy. The choice of diff erent regimens depended on diff erent era, including

carboplatin + vincristine, temozolomide alone, vinblastine alone, TPGV, PCV, etc. Patients often received more than one course of chemotherapy. Two patients underwent RT in the

early 2000 era. Only 3 patients died of disease progression (all WHO Gr I). The 5-year event free survival was 93.3 % and 100 % for Gr I & Gr II patients respectively.

Conclusion. Childhood low grade astrocytoma has relatively good prognosis even if unresectable. Low intensive chemotherapy could suppress the progression of a signifi cant

proportion of these low grade tumors and achieve long term survival. Even if it recurred, we could utilize chemotherapy again and avoid using RT on this group of patients.

A B S T R A C T N O . : P - 2 8 4

Neurological disorders in case of central nervous system (CNS) tumors

E.V. Mironova1, I.B. Mironov2, I.D. Borodina1 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Morozov Children’s City Clinical Hospital, Moscow, Russia

Key words: medulloblastoma, rehabilitation, CNS tumors

Introduction. Central nervous system (CNS) tumors are the most frequent solid neoplasms in childhood, CNS neoplasms incidence in children aged from 0 to 18 years is 3.5–4.0

per 100 000 children. The quality of life of patients with CNS neoplasms is defi ned both by pathogen itself and by a complex of complications that occur in the course of this disease.

Also the current cancer treatment methods themselves adversely aff ect patients’ organs and systems. Nervous system responds very sensitively to any problems of an organism,

therefore highly expressed neurological disorders immediately occur in the human body which prevent patients’ recovery even after the end of the therapy.

Aim. Detection and management of neurological disorders in patients with CNS tumors.

Materials and methods. Considering that 60 % of CNS tumors in children older than 3 years are localized infratentorially in posterior cranial fossa (PCF), the investigation

of 75 patients with the diagnosis “medulloblastoma” was undertaken with the use of a standard neurological examination, neurological status assessment on EDSS, ATAXIA scales.

Along with this, the possibilities of disorder management were defi ned.

Results. During examination and dynamic follow-up of 75 patients with the diagnosis “medulloblastoma” it was noted that 92 % of them have such coordination disorders

as ataxia, dysmetria and intention during coordination test; motor disorders (paresis, paralysis) were reported in 81.3 % of cases; such peripheral nervous system involvements

as polyneuropathies are detected in 41 % of patients and behavioural and neurocognitive disorders form in 89 % of children. After complex (motor, neurocognitive) rehabilitation

a positive dynamics was reported in a motor sphere in the form of intensity reduction of disorders during coordination tests, ataxia, muscular strength increase; in neurocognitive

sphere – in the form of the improvement of the results during the performance of applicable tasks – an increase of productivity, attention concentration were observed .

Conclusion. A timely detection of nervous system disorders and their complex management are an essential part of complex rehabilitation of children with CNS tumors.

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A B S T R A C T N O . : O P - 2 9 8

Preliminary results of treatment according to HIT-MED-Study Guideline 2014

in Russian cohort of patients

E. Kumirova1, S. Oserov1, L. Papusha1, E. Salnikova1, I. Borodina1, A. Samarin1, V. Yemtsova1, A. Ektova1, A. Kislyakov1,

M. Ryzhova2, A. Panfyorova1, A. Nechesnyuk1, D. Kobyzeva1, A. Usychkina1, A. Artemov1, L. Zemtsova1, A. Kazakova1,

S. Homyakova1, M. Dubrovina1, A. Pshonkin1, M. Tihonova1, A. Spirin1, S. Gorelishev2, E. Shorikov3, M. Mushinskaya4,

I. Gerbek5, E. Erega6, A. Shapochnik7, M. Belogurova8, G. Novichkova1, A. Karachunskiy1, A. Valiakhmetova2,

M. Andrianov1, G. Tereshchenko1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Scientifi c Research Neurosurgery Institute named after the academician N.N. Burdenko, Moscow, Russia; 3Children’s Regional Clinical Hospital № 1, Yekaterinburg, Russia; 4Perm Regional Children's Clinical Hospital, Russia; 5Regional Children’s Hospital,

Tomsk, Russia; 6Regional Children's Clinical Hospital named after A.K. Piotrovich, Khabarovsk, Russia; 7Orenburg Regional Clinical Oncology Center, Russia; 8City Clinical Hospital № 31 of Saint-Petersburg, Russia

Key words: brain tumors, children, chemotherapy, radiation therapy

Introduction. Federal Research Clinical Centre for Pediatric Hematology, Oncology and Immunology named after D. Rogachev (Rogachev’s Center) observe 1000–1200 children per

year with tumors of the central nervous system from all regions of Russia. Infrastructure of the center has all modern facilities of diagnosis, treatment and rehabilitation for children

with tumors of the central nervous system including standard molecular biological studies, such as MYC-amplifi cation (FISH/CISH), CTNNB1 gene mutation (Sanger sequencing).

On February 2015 after formal permission of the offi ce of HIT-MED-Study (Hamburg, Germany) the Guideline 2014 was adopted to use it in our Centre and other oncohematological

centers/departments of Russia.

Aim. Тo demonstrate the capabilities of Russian clinics to participate in international studies on the diagnosis and treatment of tumors of the central nervous system in children.

Materials and methods. From February 2015 to January 2016 38 patients (pts) with the fi rst time verifi ed primary brain tumors with the age at diagnosis from 1,9 to 14 y.o. (median

6 y.o) were included to database according to guideline’s requirements. The male:female ratio was 25:13. Resection was performed: in federal centers in 18 pts, in regional – 20.

Hystologycal type was: medulloblastoma (MB) – 21 (13 – classical MB, 5 – desmoplastic MB, 2 - MBEN, 1 – anaplastic MB), ependymoma (EP) – 10 (Grade II – 2, Grade III – 8),

PNET – 6, pineoblastoma – 1. Histology was confi rmed in all patients in reference centers (Rogachev’s Center and Burdenko Institute) M0 stage of disease was in 25 pts, M1 – 3

pts, M2 – 3 pts, M3 – 5 pts, Mx – 2 pts. Among MB-patients MYC amplifi cation was performed in 11 pts (52 %) and was positive in 1. Sanger sequencing analysis was performed

in 2 MB pts with M0R0 stage of disease and positive nuclear expression of b-cathenin, CTNNB1 mutation was detected in 1 of them. All pts received diff erent arms of therapy

according HIT-MED-Guideline 2014, among them 2 pts with MB - tandem HDCT (in Rogachev’s Center). Hyperfractionated Radiation Therapy (HFRT) and Intensive Modulated

RT (IMRT) planning and delivery techniques, which available in Rogachev’s Center, were used in 4 pts and in 27 pts respectively.

Results. All patients are alive. Finished all course of the therapy – 8 pts with total response. 1 pt with AE recurred locally. All points of the protocol requirements are fulfi llment. There

were no any SAE during the treatment.

Conclusion. The modern standard approaches of diagnosis and treatment for children with high grade neuroepithelial brain tumors including molecular biological studies are

available in Russia which can allowed taking part in international trials.

A B S T R A C T N O . : P P - 3 4 9

Results of medulloblastomas treatment in children under 5 years

E.V. Kumirova1, Е.А. Salnikova1, I.D. Borodina1, L.I. Papusha1, S.S. Ozerov1, А.Е. Samarin1, A.P. Ektova1, M.V. Ryzhova2,

А. Korshunov3, A.V. Nechesnyuk1, D.A. Kobyzeva1, O.G. Zheludkova4, O.I. Shcherbenko4, М.B. Belogurova5,

S.V. Gorbatykh6, М.V. Mushinskaya7, R.Z. Shammasov8, Е.P. Erega9, А.P. Shapochnik10, Е.V. Shorikov11, I.E. Gerbek12,

Е.V. Inyushkina13, S.A. Kulyova14, N.A. Filatova15, Е.P. Matseha16 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2 Scientifi c Research Neurosurgery Institute named after the academician N.N. Burdenko, Moscow, Russia; 3German Cancer Research Center, Heidelberg; 4Russian Scientifi c Center of Radiology and Nuclear Medicine, Moscow, Russia; 5Municipal Clinical Hospital № 31, Saint-Petersburg, Russia; 6Morozov Children’s City Clinical Hospital, Moscow, Russia; 7Perm Regional Children’s

Clinical Hospital, Russia; 8Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan; 9Regional Children’s Clinical Hospital named after A.K. Piotrovich, Khabarovsk, Russia; 10Orenburg Regional Clinical Oncological Dispensary, Russia; 11Regional

Children’s Clinical Hospital № 1, Ekaterinburg, Russia; 12Regional Children’s Hospital, Tomsk, Russia; 13Moscow Regional Oncological Dispensary, Balashikha, Russia; 14N.N. Petrov Research Institute of Oncology, St-Petersburg, Russia; 15Arkhangelsk Regional

Children’s Clinical Hospital named after P.G. Vyzhlezov, Russia; 16Trans-Baikal Regional Oncological Dispensary, Chita, Russia

Key words: children, medulloblastomas, chemotherapy, radiotherapy

Introduction. Medulloblastomas (MB) in children of younger age are one of unsolved issues in pediatric neurooncology. Prognostic-factors-based risk group stratifi cation is needed,

which will probably allow to individualize such patients treatment in the future.

Aim. To evaluate treatment results of 86 patients under 5 years (median age of 21 months) with primarily diagnosed MB.

Materials and methods. The ratio of boys and girls was 1.5:1 (60 % and 40 %, respectively). Depending on the age: in 31 follow-up – tumor resection (R0), in 49 (57 %) cases – non

radical surgery, in 3 (3.5 %) – biopsy, in 3 (3.5 %) patients the volume is unknown. Histologically: classic (class) – 43 (50 %) patients, desmoplastic (desmo) – 21 (24.4 %), large CNS

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cell/anaplastic (anapl) – 7 (8.1 %), MBEN – 1 (1.2 %), in 14 (16.3 %) patients the variant is unknown (MB NOS). Molecular genetic analysis undertaken in 31 patient: 10 – SHH

group, 5 – group 3, C-MYC-amplifi cation was in 2 cases. Depending on a disease stage: М0 – 30 (35 %) patients, М1 – 15 (17.4 %), М2 – 4 (4.6 %), М3 – 25 (29 %), М4 – 1 (1.2 %),

Мх – 11 (12.8 %). R0М0 reported in 14 (16.3 %) children. On the fi rst stage 64 (74.4 %) patients received polychemotherapy, 22 (25.6 %) patients – radiotherapy (RT). Treatment

under HIT SKK protocol was received by 77 (90 %) patients, under М-2000 protocol – by 3 patients, unprogrammed therapy – by 6 patients. Intraventricular methotrexate (MTX)

injection was received by 30 (33%) patients, intrathecal – 9 (10.5 %). High-dose chemotherapy with auto-HSCT undertaken in 15 (17.4 %) patients. RT was received by 59 (68.6 %)

patients. Positive responce to RT was achieved in 54 (91.5 %) patients, DP was reported in 5 (8.5 %) patients. On diff erent therapy stages DP was observed in 34 (40 %) patients,

14 (41 %) of which are alive. 64 (74.4 %) /86 patients are alive, 17 % continue the therapy, the rest (83 %) completed specifi c treatment

Results. Five year overall survival is а 0.42 ± 0.06 (median – 39.5 months, follow up period – from 2 to 271 months), 5-year progression free survival (PFS) – 0.60 ± 0.05

(median – 27.5 months, follow up – from 2 to 235 months). PFS in boys and girls is 0.7 and 0.49, respectively (P = 0.13). PFS depending on age: 0.2 (36 months) (P = 0,03).

PFS depending on M-stage: М0/М1/М3 – 0.62/0.59/0.53 (P = 0.25), М2/М4 – 0. PFS depending on histology: class/desmo/MBNOS/anapl = 0.7/0.55/0.58/0.29 (P = 0.15).

PFS in patients with SHH was 0.50. Depending on resection volume: PFS – 0.53 (in patients with R+) and 0.66 (R0), P = 0.28. In SHH-group patients survival without RT was 0.33,

with RT – 0.75 (P = 0.29). PFS in patients that received and did not receive intraventricular/intrathecal MTX was 0.52 and 0.50, respectively (P = 0.3).

Conclusion. Adverse prognostic factors were age (under 12 months), histological MB variant (large cell/anapl), М+ stage, presence of residual tumour, absence of RT in SHH-group

patients. For further verifi cation of treatment strategy additional studies are needed.

A B S T R A C T N O . : O P - 3 5 1

Eff ect therapy for medulloblastoma within protocol HIT-2000/2008 among children under

the age of four years

O. Zheludkova1, M. Ryzhova2, A. Melikian2, Yu. Kushel2, S. Ozerov3, E. Kumirova3, I. Borodina3,

V. Ozerova2, S. Gorbatykh4, O. Polushkina4, L. Olhova4, A. Nechesnyuk3, O. Shcherbenko1, E. Shorikov5,

A. Beznoshchenko6, R. Shammasov7, S. Kovalenko8, N. Yudina9, M. Mushinskaya10, E. Inyushkina11,

L. Privalova12, A. Matytsyn13, A. Korshunov2

1Russian Scientifi c Center for Roentgenology and Radiology; 2Scientifi c Research Neurosurgery Institute named after the academician N.N. Burdenko, Moscow, Russia; 3Federal Research Center of Pediatric Hematology, Oncology and Immunology

named after Dmitriy Rogachev, Moscow, Russia; 4Morozov Children’s City Clinical Hospital, Moscow, Russia; 5Regional Children’s Clinical Hospital № 1, Ekaterinburg, Russia; 6Ryazan Regional Children’s Clinical Hospital named after N.V. Dmitrieva, Russia;

7Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan; 8Chelyabinsk Regional Children’s Hospital, Russia; 9Voronezh Regional Children’s Hospital, Russia; 10Perm Regional Children’s Clinical Hospital, Russia; 11Moscow Regional Oncological Dispensary,

Balashikha, Russia; 12Nizhny Novgorod Regional Children Clinical Hospital, Russia; 13Tambov Regional Children Clinical Hospital, Russia

Key words: medulloblastoma, young children, chemotherapy

Introduction. Medulloblasma is the most common malignant brain tumor occuring in children. Standart treatment of medulloblastoma in older children consists of surgery,

craniospinal irradiation and chemotherapy, with overall survival approaching 85 % at 5 years for children with non-metastatic and near totally resected medulloblastoma.

The prognosis for infants and young children with medulloblastoma treated with a similar approach remains poor. The use of cranial irradiation in young children has been implicated

in learning, attention and processing speed defi cits, problems with hearing, endocrine disfunction, leukoencephalopathy and defi cits in theur physical growth.

Aim. To estimate the effi ciency of chemotherapy and chemoradiotherapy in patients with medulloblastoma under the age of four and to determine the prognostic factors.

Materials and methods. From 2009 to 2014, we analyzed 36 patients who got therapy within protocol HIT-2000/2008 after tumor resection. There were 19 (53 %) girls

and 17 (47 %) boys. The age of 5 (14 %) patients was 3–12 months, 18 (50 %) – 13–24 months, 13 (36 %) – 25–48 months. The histological variant of the tumor was: 16/3 (53 %)

DМВ/MBEN, 15 (41.5 %) CMB, and 2 (5.5 %) LCA. Stage M0 was in 25 (69.5 %) patients M+ in 11 (30.5 %). In 26 cases (72 %) underwent total resection; a residual tumor was found

in 10 patients (28 %). Chemotherapy + radiotherapy was used on 19 (53 %) patients; 17 (47 %) got only chemotherapy. Intrathecal/intraventricular Methotrexate used to 22 (61 %)

patients.

Results. PFS and OS were 0.71 ± 0.08 and 0.86 ± 0.06 respectively. The mean time of observation was 24 months, and the median time to progression was 17 months. 27 patients

completed their treatment, 9 were diagnosed with recurrence/metastases, and 4 died of PD. PFS/OS was 0.88/1.0 for patients aged 25–48 months, 0.62/0.77 for 13–24 months

and 0.60/0.80 for 3–12 months. There were no signifi cant diff erences in PFS/OS depending on the histological variant of medulloblastoma: 0.61/0.85 for DMB and MBEN, 0.79/0.86

for CMB, and 1.0/1.0 for LCA. Patients with stage M0 had PFS/OS 0.74/0.95 and for M+ 0.64/0.65. PFS/OS among patients with M0R0 were 0.64/0.92; for М0R+ 0.89/1.0.

PFS for patients who received intrathecal/intraventricular MTX was 0.79 and 0.63 who didn’t. This was also true for DMB/MBEN: 0.75 and 0.43, respectively. PFS was 0.67 among

patients who got radiotherapy and 0.76 among those who didn’t.

Conclusion. Multiagent chemotherapy is effi cient in MB patients under the age of 4. A low survival rate was observed among patients under 1 year and with metastases.

The completeness of the surgical resection and the use of radiotherapy did not aff ect the results of treatment. The best survival rate was found among patients who got regional

therapy with MTX, including those with DMB/MBEN.

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A B S T R A C T N O . : P - 3 6 4

Results of treatment of intracranial AT/RT in children

O. Zheludkova1, M. Ryzhova2, L. Shishkina2, A. Melikyan2, Yu. Kushel2, L. Olhova3, O. Polushkina3, S. Gorbatykh3,

E. Kumirova4, I. Borodina4, E. Inyushkina5, V. Ozerova2, M. Mushinskaya6, N. Popova7, L. Privalova8, R. Shammasov9,

O. Shcherbenko1, E. Abbasova1, A. Kryanev1, O. Chulkov10, N. Yudina11, A. Korshunov2, A. Matytsyn12, I. Vorotnikov13

1Russian Scientifi c Center for Roentgenology and Radiology; 2Scientifi c Research Neurosurgery Institute named after the academician N.N. Burdenko, Moscow, Russia; 3Morozov Children’s City Clinical Hospital, Moscow, Russia; 4Federal Research

Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 5Moscow Regional Oncological Dispensary, Balashikha, Russia; 6Perm Regional Children’s Clinical Hospital, Russia; 7Volgograd Regional Clinical

Oncological Dispensary № 1, Russia; 8Nizhny Novgorod Regional Children Clinical Hospital, Russia; 9Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan; 10Children’s Regional Clinical Hospital, Krasnodar, Russia; 11Voronezh Regional Children’s

Hospital, Russia; 12Tambov Regional Children Clinical Hospital, Russia; 13Center of New Medical Technologies, Novosibirsk, Russia

Key words: AT/RT, children, treatment, malignant tumor

Introduction. Atypical teratoid/rhabdoid tumors are highly malignant central nervous system (CNS) tumors. As compared with other malignant CNS tumors, their biological

behavior is particularly aggressive, but patients may benefi t from an intensifi ed treatment.

Aim. To determine prognostic factors in patients with AT/RT.

Materials and methods. We have evaluated the prognostic factors in 43 patients with AT/RT. Most patients were younger than 3 years old (28, 65 %), and 15 patients (35 %)

were above 3 years. The boys and girls were 20 and 23, respectively. The tumor was infratentorial in 21 patients (48.8 %); 2 patients (4.7 %) had infratentorial and renal tumors;

and 20 patients (46.5 %) had supratentorial tumors. Stage M0 was in 24 patients (55.8 %); 11 patients (25.6 %) had metastases or detected tumor cells at diagnosis; and the stage was

not precisely determined for 8 patients (18.6 %).Treatment according to protocol ATRT-2006 was administered to 24 patients (55.8 %); protocol CWS, to 8 patients (18.6 %); HIT-SKK,

to 4 patients (9.3 %); and 7 patients (16.3 %) got off -protocol treatment.

Results. 13 patients (30.2 %) are alive, and 30 (69.8 %) have died: 26 due to disease progression, 4 due to chemotherapy toxicity. The PFS was 30 ± 0.06%, and the OS was 38 ± 0.06%.

The median survival time was 18 months; and the median observation time was 14 months (range 2 to 89 months). The survival rate was signifi cantly higher among patients over

3 years old in comparison with younger patients: 53 % and 14 %, respectively (P = 0.004); among patients after total tumor resection in comparison with subtotal or partial resection:

55 %, 31 %, and 12 %, respectively (P = 0.015); among patients who got radiotherapy in comparison with those who did not: local radiotherapy, 50 %; craniospinal radiation, 35 %;

no radiotherapy, 0 % (P = 0.033); among patients with stage M0 in comparison with stage M+: 37 % and 0 %, respectively (P = 0.007). Therapy according to the ATRT-2006 protocol

led to better survival rate (43 %) in comparison with protocols CWS (12 %) and HIT-SKK (18 %), P = 0.01.

Conclusion. The factors that aff ected the prognosis were the patient’s age, the extent of the surgical resection, the chemotherapy program, the use of radiotherapy, and the presence of metastases.

A B S T R A C T N O . : P - 3 6 6

Results of treatment for childhood with anaplastic ependymoma

O. Zheludkova1, Yu. Kushel2, A. Melikyan2, S. Ozerov3, L. Shishkina2, M. Ryzhova2, A. Kislyakov3, I. Borodina3,

V. Emtsova3, S. Gorbatykh4, L. Olhova4, L. Privalova5, M. Mushinskaya6, N. Yudina7, N. Popova8, V. Ozerova2,

O. Shcherbenko1, E. Abbasova1, A. Kryanev1, P. Pankov1, M. Salpagarov1, A. Nechesnyuk3, E. Inyushkina9, N. Filatova10,

Yu. Kozel’11, O. Chulkov12, L. Minkina13, O. Polushkina4, E. Erega14, Kh. Tsirenova15, R. Shammasov16, A. Korshunov2

1Russian Scientifi c Center for Roentgenology and Radiology; 2Scientifi c Research Neurosurgery Institute named after the academician N.N. Burdenko, Moscow, Russia; 3Federal Research Center of Pediatric Hematology, Oncology and Immunology

named after Dmitriy Rogachev, Moscow, Russia; 4Morozov Children’s City Clinical Hospital, Moscow, Russia; 5Nizhny Novgorod Regional Children Clinical Hospital, Russia; 6Perm Regional Children’s Clinical Hospital, Russia; 7Voronezh Regional Children’s

Hospital, Russia; 8Volgograd Regional Clinical Oncological Dispensary № 1, Russia; 9Moscow Regional Oncological Dispensary, Balashikha, Russia; 10Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhlezov, Russia; 11Rostov Cancer Research

Institute, Rostov-on-Don, Russia; 12Children’s Regional Clinical Hospital, Krasnodar, Russia; 13Regional Children’s Clinical Hospital № 1, Vladivostok, Russia; 14Regional Children’s Clinical Hospital named after A.K. Piotrovich, Khabarovsk, Russia; 15Children’s

Republican Clinical Hospital, Ulan-Ude, Republic of Buryatia; 16Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan

Key words: anaplastic ependymoma, children, treatment, oncology

Introduction. Ependymoma is a relatively rare brain tumor which occurs about 9 % in children and 3 % in adults out of all primary brain tumors. In children 90 % of ependymomas

develop in the brain. The treatment option for ependymoma is surgical resection followed by radiotherapy, although there are no randomized prospective studies available yet.

Presently there are no standards treatment for ependymoma.

Aim. To evaluate the outcome of children with anaplastic ependymoma after surgery, chemotherapy and radiotherapy and to identify prognostic factors for survival.

Materials and methods. We evaluated the treatment results for 169 children with AE. Median age was 4 y.o. Most patients were over 3 y.o (119; 70.4 %). There were 104 boys

(61.5 %) and 65 girls (38.5 %). Infratentorial tumors were in 81 pts (48 %); supratentorial, in 82 (48.5 %), and 6 pts (3.5 %) had the tumor in the spinal cord. 118 pts (70 %) had stage

M0; 14 (8 %) had metastases or detected tumor cells; for 37 patients (22 %), the stage was not known. 78 pts (46 %) received chemo- and radiotherapy according to protocol HIT

2000/2008; 57 (34 %) received only radiotherapy after the surgical operation; 25 (15 %) patients got chemotherapy; and 9 (5 %) pts were treated using only surgical intervention.

Most patients underwent total (n = 70) or subtotal (n = 91) tumor resection; for 8 (5 %) patients, the extent of the resection was not evaluated. CNS

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Results. 3-year PFS 0.47 ± 0.05, 5-year PFS 0.32 ± 0.05, median PFS 32 months (2 to 34 months). 3-year survival was better in children over 3 in comparison with younger

than 3 y.o: 0.50 and 0.36, respectively. 3-year survival among girls was 0.36; among boys, 0.22 (P = 0.19). 3-year PFS were better for supratentorial tumors in comparison with

infratentorial: 0.54 and 0.39, respectively (P = 0.19). Survival results were better among patients with stage M0 in comparison with stage M+: 0.50 and 0.39, respectively. In the case

of total tumor resection, the 3-year survival was better than in the case of subtotal resection: 0.42 and 0.22, respectively (P = 0.44). PFS were the same among patients who received

chemoradiotherapy or only radiotherapy: 0.53 and 0.50, respectively. Among patients who got chemotherapy after tumor resection, the 3-year PFS were 0.19; after surgery only, all

patients had recurrence (P = 0.0002).

Conclusion. The patient’s age, tumor location, the volume of surgical treatment and the M stage was seriously aff ected on the prognosis. Better survival rates observed on patients

older than 3 years, with the supratentorial localization, total removal of the tumor and without metastasis. Chemotherapy does not improve the results of treatment for anaplastic

ependymoma.

A B S T R A C T N O . : P - 3 7 8

Radiation therapy and Nimotuzumab in children and adolescents with brainstem gliomas:

a 5-year institutional experience

Migdalia Perez, Jose Alert, Jesus Reno, Mariuska Forteza

National institute of Oncology and Radiobiology of Cuba, Havana

Key words: brainstem gliomas, Nimotuzumab, children, adolescents

Introduction. Brainstem gliomas (BSG) are central nervous system (CNS) tumors with a median survival time of approximately 9 months. Up to now chemotherapy has not shown

to improve survival in these patients. The outcome of radiation therapy (RT) in combination with Nimotuzumab is shown in the present report.

Aim. Appoint the global over-life utilizing the monoclonal antibody was ours objective.

Materials and methods. 28 children and adolescents were included between Jan/2009 and Dec/2012 with the diagnosis of BSG and follow-up till January 2015. All patients

had diff use infi ltrative pontine gliomas (DIPG) and were irradiated with a dose ranging from 54 to 59.6 Gy at the National Oncology and Radiobiology Institute in Havana, Cuba.

Three patients were planned with IMRT and 25 with 3D Conformal RT. Nimotuzumab was indicated at the dose of 150 mg/m2 weekly during the time of RT treatment, then every

15 days during 8 weeks and fi nally

monthly for 1 year. Univariate and multivariate Cox regression models and Kaplan–Meier survival were analyzed to evaluate the survival.

Results. Median age at diagnosis was 7 years (range 3–18 years old), median overall survival was 17.3 months (95 % CI 14.0–20.5 ) since the beginning of the treatment

and the accumulated survival at 5 years of treatment was 42.9 %. There was balance in sex, age and dosage of RT in the population. Addition of Nimotuzumab to RT was safe.

Conclusion. The combination of radiotherapy and Nimotuzumab were well tolerated in this brainstem tumours patient’s series.

A B S T R A C T N O . : P - 3 9 1

CNS Tumors in Children of Tatarstan RepublicV.S. Ivanov, E.F. Fatyhova, E.N. Grishina, A.G. Gazizov, D.I. Abdullin, V.V. Frolov

Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan

Key words: children, CNS tumors

Introduction. CNS tumors is the second most common cancer in children.

Aim. To analyze the results of combined therapy of CNS tumors in children and adolescents younger than 18 years treated in Tatarstan during the last 10 years.

Materials and methods. From 2004 to 2015 two hundred nineteen patients aged 1 month to 17 years (24 % were children younger than 3 years) with CNS tumors underwent

surgical treatment in Neurosurgery Department. More than half (57 %) of the patients were males.

Results. At hospital admission initial neuroimaging (CT and contrast MRI) was performed in all cases. Total tumor excision was performed in 73.6 %, subtotal resection

in 16.6 %, partial resection in 8.3 % and tumor biopsy in 1.4 % of cases. Post-surgical mortality was 1.01 % (both patients deceased in 2004). Since 2005 there had been no

post-surgical mortality. Pathology verifi cation of tumor was done in all cases. In more than half of the cases confi rmatory review was performed in reference laboratories (NIINH named

after N.N. Burdenko, FRCC named after Dmitry Rogachev). After histological verifi cation of tumors 189 patients underwent combined treatment in Department of Oncohematology

of the Children’s Republican Clinical Hospital.

Conclusion. Survival after combined treatment varied in children with histologically diff erent tumor types. In patients with low grade gliomas survival was 100 %. In patients with

embryonal tumors it was 50 %. Overall survival in patients with CNS tumors after combined therapy was 66 %.

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A B S T R A C T N O . : P - 3 9 4

Evolution of brain tumours treatment results in childhood during 20 years

in Voronezh region

G.V. Trubnikova1, T.V. Stepanova2, A.M. Pozdniakov2, A.P. Shvyrev2

1Voronezh Regional Children’s Clinical Hospital № 1, Russia;2Voronezh State Medical University named after N.N. Burdenko, Russia

Key words: brain tumours, treatment, children, chemotherapy

Introduction. Intracranial neoplasms are the most common solid tumours in childhood. There are made up of a variety of histological subgroups, comprising embryonal tumours.

Treatment strategy includes surgery, irradiation, and diff erent chemotherapy regimens. The outcome depends on the extent of surgery, histological type of the tumours.

Aim. We compare the results of the chemotherapeutic treatment in children with brain tumours during two 10-year periods since 1994.

Materials and methods. In a retrospective, longitudinal study, we reviewed patients with brain tumours, treated with craniospinal irradiation and diff erent chemotherapeutic

regimens, during two periods: from 1994 to 2003 and from 2004 till 2013 in Voronezh region. Data collection included age of diagnoses, length of follow up, extent of resection, stage

of the tumours and histology.

Results. Since 1993 year 25 children (male/female – 16/9) with Brain Tumours were diagnosed in Voronezh children’s centre of oncoheamatologyl. We observed 15 children with

primary tumours and 10 relapsed patients. The median age was 6.5 years. Histologically there were astrocytoma, medulloblastoma, PNET, ependymoma, angioreticulosarcoma,

others. All were treated with surgery, irradiation and chemotherapy according to the HIT-91 protocol. Three (20 %) patients with primary tumours survived, in one of them the

second malignant disease was found. 12 (80 %) patients died. The outcome of the relapsed patients was fatal. Since 2004 the cohort includes 53 patients (male/female – 30/23) with

primary brain tumours, verifi ed in Moscow N.N.Burdenko Neurosurgery Institution. Average duration of follow up was 10 years. The median age was 7.5 years. Tumours were identifi ed

as medulloblastoma in 17 (32 %) patients (M0 stage in 12, M1, M2, M3 – in 5); anaplastic astrocytoma and glioblastoma in 8 (15 %). Low-grade astrocytomas was identifi ed

in 4 (7.6 %), anaplastic ependymoma in 5 (9.5 %), PNET in 4 (7.6 %), ATRO in 3 (5.7%) patients, pineablastoma in 1 (1.9 %), hystiocytic sarcoma in 1 (1.9 %), germinoma in 2 (3.8 %).

Brain stem tumours in 8 (15%) were not verifi ed. We used treatment regimes according to HIT-2000, HIT-SKK, M-2000, ATRO- protocol, temodal cycles, regimen avastin plus

irinothecan. CR was registered in 10 (58.9 %) patients with medulloblastoma, 1 child with ATRO, in 2 (40 %) patients with anaplastic ependymoma; SD-in 1 (25 %) patient with PNET.

The progression of the disease and death were in 7 (41.1 %) patients with medulloblastoma, in 6 (75 %) with anaplastic astrocytoma and glioblastoma (median of follow up was

10.6 months), in 2 (66.6 %) with ATRO (median of survival was 12 months), in 2 (50 %) patients with PNET (median of follow up is 36 months), in 1 patient with hystiocytic sarcoma,

in all patients with brain stem tumours (median of follow up is 11 months).

Patients with low grade astrocytoma relapsed after the irradiation and treated with the protocol for low grade gliomas. CR was registered in 1, SD – in 3 patients (median of survival

69.5 months).

Conclusion. Treatment results of brain tumours in the last decade has nearly better evolution. The combination of RT and chemotherapy with HIT-2000 protocol in patients

with medulloblastoma provided encouraging outcomes The treatment of malignant BT in children is a problem to solve.

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A B S T R A C T N O . : P P - 2 0 4

Prediction of course of ITP in children

T.A. Uglova

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: ITP, children, prediction

Introduction. ITP is the most common acquired bleeding disorder of childhood. The prognosis of patient’s ITP course at time of diagnosis will allow to individualize the therapy.

Aim. To develop the prognosis of course of de novo ITP at children at time of initial diagnosis

Materials and methods. We examined 264 children at age 1–18 years with de novo ITP. There were 147 children with acute form of disease (group I) and 117 children developed

chronic form of disease later (group II). The use of logistic regression analysis for the determination of independent variables showed that virus prodrome and dynamics of platelets

correlate with the acute course of the disease.

Results. Analysis of phenotype of lymphocytes in the peripheral blood at children of group I and II, conducted at time of diagnosis of de novo ITP, showed that the level of CD3+

lymphocytes were signifi cantly lower (p of CD3+CD16+CD56+cells was signifi cantly higher (pel of platelets at children of group I and the level of CD3+CD16+CD56+ lymphocytes

(0.78; рcant diff erences in the presence of three main isotypes of serum immunoglobulines (Ig) G, M, A at children of the analyzed groups. Another important fact is the signifi cant

increase of concentration of IgE at children of group II in comparison to group I (median 103.4 and 20.99 respectively, P = 0.000006). IgE marks the imbalance of immune-regulatory

subpopulations and disturbances of immune-regulatory mechanisms of T-cells. This could mean that the progress of autoimmune destruction of platelets at children of group II occurs

with the involvement of IgE-mediated reactions because the majority of patients did not have allergological anamnesis (the absence of anamnestic data about the clinical indications

of allergic diseases and no sign of the presence of such diseases among the relatives. The switching of B-cells to synthesis of IgE is infl uenced by the T-helpers 2, and especially

by cytokines as well as result of interaction with T-cells. Using decision tree technique we discovered the following algorithm of prediction of course of disease at children of less

than 6 years old and more than 6 years old (prediction power 87 % and 83 % respectively).

Conclusion. Prediction of children with de novo ITP can be summarized as follows. Acute ITP: at boys > 6 years old with initial IgE 6 years with initial IgE 70 ME/l and IgG 12 g/l;

at children of 1–6 years old with initial IgE 12 %; at girls of 1–6 years old with initial IgE 35 ME/l and CD19+ cells 12 %; at children witinitial IgE 12 % and platelet count on day 28 less

than 150 × 109/l; at children with initial IgE 70 ME/l; at children of 1–6 years old with initial IgE > 35ME/l; at girls older than 6 years old with initial IgE 12g/l; at boys of 1–6 years old

with initial IgE and CD19+ cells platelet count on day 28.

NON-MALIGNANT HEMATOLOGY

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A B S T R A C T N O . : P - 2 0 8

The experience of propranolol use in patients with Kasabach–Merritt syndrome

L.A. Hachatryan1, M.V. Panina1, D.M. Nikolaeva1, A.A. Maschan1, G. Henze2

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Charité Universitätsmedizin Berlin, Berlin, Germany

Key words: Kasabach–Merritt syndrome, haemangioma, thrombocytopenia, consumption coagulopathy

Introduction. Kasabach–Merritt syndrome (KMS) is a rare, life-threatening disease, characterized by vascular tumor (kaposiform hemangioendothelioma or “tufted” angioma) and

coagulopathy and thrombocytopenia consumption associated with such tumor. KMS is characterized by high mortality (up yo 30 %), caused by haemorrhagic complications, invasive

tumor growth and compression of vital organs. Nowadays for KMS treatment several therapeutic approaches are off ered: glucocorticosteroids (GCS), alpha-interferon (IF), vincristine

(Vcr), cyclophosphamide, rapamycin. None of them is universally eff ective and is associated with serious side-eff ects. Surgical treatment approach is rarely possible due to a large

size of the tumor and infi ltrating growth. Search of an eff ective and safe agent is a vital issue. Nonselective propranolol (PP) beta-blocker is considered as one of potentially eff ective

medicines. The basis of its mechanism of action: vasoconstriction, VEGF (vascular endothelial growth factor ) and bFGF (fi broblast growth factor) blocking – induced endothelial

cells proliferation, matrix metalloproteinase and interleukin 6 (proangiogenic cytokine), apoptosis induction. However, the effi cacy of PP is proved only in treatment of infantile

hemangioma.

Results. We present clinical cases. № 1. Patient B.K. (21 days) has been ill since his birth. Diagnosis: KMS, haemangioma of anterior abdominal wall and perineum. The blood

analysis (BA): thrombocytes (Tr) – 7 х 109/l, fi brinogen (F) – 0,8 g/l, D-dimer (DD) – 43.381 ng/ml. Therapy: Prednisolone (GCS) dose – 5–8 mg/kg/day since birth (on b/g) – without

eff ect; PP – 2 mg/kg/day. Normalization of hemogram and hemostasis indices and reduction of mass sizes by 2 times were in 100 days. Remission was achieved in 14 months.

№ 2. Patient L.A. (2 months) has been ill since his birth. Diagnosis: KMS, haemangioma of lower-right-hand. BA: Tr – 6 х 109/l, F – 0.5 g/l, DD – 10.651 ng/ml. Therapy: GCS in

the form of monotherapy combined with Vcr (without eff ects) and IF (minimal response: Tr – 30 х 109/l). Complication: virus pneumonia. PP dose – 2 mg/kg/day. Normalization of

hemogram and hemostasis indices and reduction of mass sizes by 2 times were in 100 days. Remission was achieved in 6 months. № 3. Patient T.N. (12 months) has been ill since

his birth. Diagnosis: KMS, haemangioma of lower-right-hand, perineum, anterior abdominal wall. BA: Tr – 11 х 109/l, F – 1.3 g/l, DD – 5016 ng/ml. Therapy: GCS (on b/g)

dose 3–5 mg/kg/day during 12 months – transient increase of Tr up to 120 х 109/l without tumor size reduction. Complication: bilateral pneumonia. 2 courses of laser therapy (on b/g) –

transient increase of Tr up to 240 х 109/l. PP dose 2–3 mg/kg/day. Normalization of hemogram and hemostasis indices (Tr – 200 х 109/l; F – 2.65 g/l), tumor size reduction by 1/3

were in 24 days. Total remission was in 12 months.

Conclusion. It is the fi rst time when the protocol for infantile haemangioma treatment in patients with KMS was used in our country on the basis of our clinic. PP showed a range

of advantages in comparison with a “standard” therapy. High effi cacy: hematological response was achieved on average in 30 days; tumor size reduction on average by 2 times

was in 90 days, remission was in 6–12 months. Safety: no PP-associated side-eff ect or complication was registered. Easy to use – only heart rate, blood pressure monitoring and

electrocardiogram are needed.

A B S T R A C T N O . : P - 2 2 8

The case of successful treatment of right atrium thrombosis

and right internal jugular vein in newborn

A. Kostromin1, R. Shammasov1, I. Nurmeev2, D. Osipov1, I. Osipova1

1Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan; 2Kazan State Medical University, Republic of Tatarstan

Key words: newborn, thrombosis, heart, removal

Introduction. Venous thrombosis in newborns is rare situation. Usually they are catheter-related cases. There is no a standard treatment tactic. There are no reported cases of

thrombosis of heart cavities in newborns. We are reporting a case of successful treatment of right atrium thrombosis in newborn.

Aim. To demonstrate a case of diagnostics and treatment of right atrium thrombosis in newborn.

Materials and methods. Reported case – full-term newborn boy aged 26 days at time of revealing of thrombosis. History: neonatal septicemia, multiply abscesses of legs

and hands, pneumonia and right shoulder joint arthritis. Ultrasound investigation had demonstrated thrombus in right atrium, consisting of two interconnected halves, one of them

7 × 8 × 7 mm was prolapsed into right ventricle through tricuspid valve. Fixing point was at the base of inferior vena cava. There was a high risk of rupture and thromboembolism

and thrombus was removed with surgical procedure with extracorporeal circulation. Procedure and post-operative period – uncomplicated. Heparin sodium was administered.

On the 10th day thrombosis of right internal jugular vein was revealed, covering intravenous catheter. From 15th day anticoagulant therapy was changed to Nadroparin calcium,

subcutaneous injections.

Results. Follow-up ultrasound after 1 month and 3 months after beginning of treatment had demonstrated clearance of jugular vein, absence of clots inside heart. Anticoagulant

treatment stopped after 1 month.

Thus, there is a case of successful treatment of thrombosis of heart and deep veins in newborn.

Conclusion. Thrombosis of heart cavities is a rare and dangerous condition, it is requiring urgent actions; presence of risky thrombus inside heart is indication for surgical removal

of thrombus.

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A B S T R A C T N O . : P P - 2 3 6

Visceral leishmaniasis in the practice of pediatric oncohematologistA.M. Chililova1, I.M. Yunusova1, G.A. Gadjibalaeva1, M.E. Dubrovina2, P.S. Umarova1

1Children’s Republic Clinical Hospital named after N.M. Kuraev, Republic of Dagestan, Russian Federation; 2Federal Scientifi cand Clinical Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: leishmaniasis, Leishmania donovani

Introduction. Leishmaniasis is an infectious disease caused by intracellular parasites of the genus “Leishmania”. Vectors are mosquitoes. This disease is spread over vast areas

of subtropical and tropical zone (Middle East, Central Asia, Eastern and Central Africa). There are visceral and cutaneous clinical forms. The incubation period for the visceral form

of leishmaniasis is from 3 weeks to 10–12 months. Leishmania parasites in the human cells of the reticuloendothelial system and cause hypertrophy of reticuloendothelial cells

of the spleen, liver, bone marrow, lymph nodes or other lymphoid tissues, and therefore, patients with this disease often directed to pediatric hematologists and oncologists.

Aim. Description of a clinical case of visceral form of leishmaniasis.

Materials and methods. Parents of the child aged 1 year and 2 months, living in rural area, appealed to the local pediatrician with complaints about the deterioration in the form of

febrile fever, weakness and lethargy. Antibiotic therapy was initiated without the improvement of the clinical condition. The complete blood count showed signifi cant changes and the

child was referred to DRKH named after N.M. Kuraev (Makhachkala) with a diagnosis of “hematological malignancies?” for further examination and determination of tactics of treatment.

At the time of the admission the performance status was poor due to the severe symptoms of intoxication, febrile fever. Noteworthy pronounced pallor of the skin, hyperplasia

of the lymph nodes in all groups, hepatosplenomegaly (liver – at 3 cm from under the costal arch, the spleen – 4 cm). The complete blood count found changes in the form

of 3-lineage aplasia of hematopoiesis: Hb – 58 g/l, the PLT – 92 000/mcl, granulocytes – 738/mcl, erythrocyte sedimentation rate – 62 mm/h. Blood serology was negative for human

immunodefi ciency virus, hepatitis B virus, cytomegalovirus, herpes simplex virus, Epstein–Barr virus.

Bone marrow puncture was done. The myelogram showed medium bone marrow cellularity and polymorphic composition. The blasts were detected. Myeloid lineage narrowed.

Erythroid lineage was hyperplastic. Megakaryocytes, active and inactive. Increased % of lymphocytes. Leishmania was found in macrophage cells and intracellular. Morphology -

violet color nucleus with oval cytoplasm. Leuko-erythroblastic ratio is 1.04: 1. Bone marrow neutrophil index – 0.33. maturation index of red blood – 0.87. Formolovaya test was

positive. Based on clinical and laboratory data the diagnosis was made of visceral form of leishmaniasis, acute course. Supportive therapy was conducted in the department.

Bone marrow smears were revised in FSCC PHOI named after Dmitry Rogachev (Moscow) and the Center for Infectious Diseases (Moscow). The diagnosis was confi rmed. Due to the

nature of the disease the child was sent to the Center for childhood Infectious Diseases (Baku), where there is a specialized department for the treatment of leishmaniasis. The child

was treated using combined specifi c therapy including 5-valent surma, amphotericin B, allopurinol. The patient’s condition was improved.

From 2012 to 2015 in the Republic of Dagestan 3 children have been identifi ed with this diagnosis. The diagnosis in all cases was laboratory confi rmed by the detection of Leishmania

donovani in the bone marrow punctate.

Results. Currently, the child is in remission, continues to be observed.

Conclusion. This case illustrates the case of non-endemic infection in Russia. Pronounced changes in the hematopoiesis in this group of patients, usually associated with late

diagnosis of the disease and, consequently, the late administration of a specifi c therapy.

A B S T R A C T N O . : O P - 2 4 5

Serum interleukin-10 level in childhood immune thrombocytopenia and its role

in predicting the clinical course – a prospective case control study

Vinod Gunasekaran, Nita Radhakrishnan, Veronique Dinand, Anupam Sachdeva

Sir Ganga Ram Hospital

Key words: immune thrombocytopenia, serum interleukin-10, predictive value

Introduction. Majority of children diagnosed with immune thrombocytopenia (ITP) have a self-limiting course with excellent prognosis. However, a small proportion of children may

have a protracted course, leading to signifi cant morbidity, irrespective of modes of treatment used at diagnosis. Various studies have been performed so far to predict the long-term

outcome of ITP, depending on the variables available at presentation. No single clinical or laboratory marker at diagnosis is found to accurately predict the course of childhood ITP.

Serum interleukin-10 (IL-10) is an anti-infl ammatory cytokine produced by various white cells upon infl ammatory stimulus.

Aim. The aim of our study is to determine the signifi cance of serum IL-10 level at diagnosis in children with immune thrombocytopenia as a marker in determining the long term outcome.

Materials and methods. Twenty children (aged 1–18 years) newly diagnosed as ITP (based on clinical picture, blood counts and bone marrow evaluation) were prospectively

enrolled for a period of 1 year (November 2014 – October 2015). Clinical and laboratory details were recorded. Serum IL-10 level was measured prior to initiating treatment. Treatment

decisions were taken by the treating hematologist. Children were prospectively followed up at 3 months, 6 months and 1 year intervals with bleeding history, blood counts and

treatment details (with a median follow-up of 7.5 months). Outcome (acute or non-acute) was classifi ed according to guidelines laid by the International Working Group on ITP

(Vicenza Consensus Conference). Serum IL-10 level was also measured in 20 age-matched healthy controls.

Results. Among 40 children, there were 23 (57.5 %) males and 17 (42.5 %) females. Cases and controls were matched for sex (M:F ratio 12:8 vs. 11:9, P = 1.0) and age

[median age (IQR) 4.5 (3–6.75) vs. 4 (2–7) years, P = 0.624]. There were 10 acute ITP cases (i.e. disease duration ≤ 3 months). The median IL-10 level (IQR) was higher in ITP cases

than in controls [8.95 (4.52–13.38) vs. 3 (0.94–9.04) ng/ml, P = 0.005). Acute ITP cases [11.44 ng/ml (6.26–21.3)] demonstrated higher median IL-10 levels than non-acute cases

[7.28 ng/ml (3.67–10.83)], but statistical signifi cance could not be demonstrated (P = 0.151).

Conclusion. Serum IL-10 at diagnosis was signifi cantly higher in children with ITP as compared to age matched controls suggesting an underlying infl ammatory state. Children with

an acute illness had higher levels of serum IL-10 at diagnosis as compared to those with protracted course, suggesting that an anti-infl ammatory cytokine response might shorten

the disease duration. However a larger study is required to demonstrate the optimal IL-10 cut-off and its predictive value at the time of diagnosis.

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A B S T R A C T N O . : O P - 3 4 2

Coombs test – easy diagnosis of diffi cult diseases

N.E. Sokolova, N.I. Vaynyunskaya

Saint-Petersburg City Children Hospital № 1, Russia

Key words: hemolysis, purpura, Combs test, prognosis of severity of disease

Introduction. In recent years, more common diseases occur, which are caused by the immune processes. Direct antiglobulin test is designed to detect incomplete erythrocytic

antibodies, fi xed on erythrocyte membrane. Since the middle of last century, this method is the “gold standard” for detection of antibodies of class IgG and C3 component of complement

fi xed on the erythrocyte surface. Once sensitization has already occurred in the body and the subsequent addition of antiglobulin serum causes agglutination of sensitized cells.

Most often the antibodies elicited by direct Coombs test, are not associated with the specifi ed antigen. Most often IgG (typically IgG1 and IgG3) are detected on the erythrocyte

membrane, that can activate complement. Traditionally, this test was used for the diagnosis of immune hemolytic anemia, which developed as a result of exposure to incomplete heat

antibodies. However recently, positive results of the test have been observed in various pathological conditions caused by the adsorption of antibodies to erythrocyte membrane surface.

Aim. Primary screening diagnostics of various diseases of the blood and connective tissue by direct antiglobulin test.

Materials and methods. Patients of the department of pediatric hematology of the Сity childrens hospital № 1 in St. Petersburg in 2015. The patients’ age were from 1 year to 14 years;

boys – 12, girls – 8. The reason for the hospitalization of 10 patients was the development of severe anemia, with three children arrived with the clinic of acute thrombocytopenic

purpura, 3 patients suff ered from thalassemia, 3 patients complained of lymphadenopathy, 1 child with clinical hemorrhagic vasculitis. Complete work-up was done including Coombs test.

Results. In a survey of all the children positive direct Coombs test has been found due to the presence on the erythrocyte membrane both IgG antibodies and C3d component

of complement. In conjunction with the corresponding clinical picture all patients had initial diagnosis transformation and verifi cation of this disease. The fi rst 10 patients were

suff ering from acute immune haemolytic anemia due to incomplete heat antibodies. Children entered the clinic with acute thrombocytopenic purpura, subsequently was diagnosed

with Evans syndrome, thalassemia patients developed hyperhemolisis syndrome, children with lymphadenopathy was diagnosed with autoimmune lymphoproliferative syndrome,

and in the child with the clinic of hemorrhagic vasculitis the onset of systemic lupus erythematosus was detected. Detection of primary positive Coombs reaction allowed to perform

extended further examination and, where necessary, reduce the time of diagnostic search.

Conclusion. Direct antiglobulin test is a simple and aff ordable method which allowed to suspect, and in some cases, to diagnose severe systemic immune diseases. It is necessary

to further study Coombs test as a prognostic criterion in the course and treatment of hematological diseases.

A B S T R A C T N O . : P P - 3 5 8

Phenotypic variability in ELANE–related neutropenia from mutated Ala57 residue

Chang-Hun Park, Silvia Park, Yae-Jean Kim, Sun-Hee Kim, Hee-Jin Kim

Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine

Key words: ELANE-related neutropenia, Ala57Asp, mutation, cyclic neutropenia

Introduction. ELANE-related neutropenia includes severe congenital neutropenia and cyclic neutropenia from a germline mutation in the ELANE gene with an autosomal dominant

inheritance. Both are clinically characterized by recurrent fever, skin and oropharyngeal infl ammation. Clinical manifestations are milder in cyclic neutropenia than in severe

congenital neutropenia.

Aim. We herein describe a novel ELANE mutation in a Korean patient with neutropenia.

Materials and methods. The patient was a 20-year-old man who presented with episodes of febrile neutropenia and cervical adenopathy. He had a history of self-limiting febrile

episodes associated with pain and swelling in the buccal area since 7 years of age. The initial CBC revealed a WBC count of 1.98 × 109/L with an absolute neutrophil count of 0.26 × 109/L.

Serial counts of absolute neutrophils revealed a cyclic pattern, indicating cyclic neutropenia. Bone marrow study revealed maturation arrest in the granulocytic lineage. Direct

sequencing analyses was performed to cover coding exons and fl anking intronic sequences of the ELANE gene.

Results. We identifi ed a novel heterozygous missense variant in exon 2 of ELANE: c.170C>A (p.Ala57Asp). Bioinformatics analyses and reference database search according

to the new ACMG/AMP variant classifi cation guidelines indicated the variant is pathogenic. The Ala57 residue of ELANE is a mutation hotspot with previously reported missense

mutations Ala57Ser, Ala57Thr, and Ala57Val, which is explained by the CpG site of the codon. Of note, previous cases with mutated Ala57 were severe congenital neutropenia without

evidence of a cyclic pattern.

Conclusion. Thus, the present case demonstrates a phenotypic variability in ELANE–related neutropenia from mutated Ala57 residue.

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A B S T R A C T N O . : O P - 4 8 1

Castleman’s disease in pediatric patients

A.L. Kozlova, V.E. Burlakov, D.S. Abramov, N.V. Miakova, D.N. Konovalov, A.Yu. Shcherbina

Federal Research and Clinical Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Introduction. Castleman’s disease is a rare lymphoprolipherative disorder that is characterized by lymph node hyperplasia. There are two major forms: localized (or unicentric)

and systemic (or multicentric). Another way of classifying the disease is based upon pathomorphological fi ndings in the aff ected lymph nodes: hyaline-vascular type, plasma cell type

or mixed type. Though multiple hypothesis have been proposed, the pathogenesis of the disease remains unknown. The age of onset can diff er greatly from infancy to adulthood.

Except for lymph node hyperplasia, patients with Castleman’s disease can also develop such symptoms as anemia, fatigue, fever and pain in the area of the aff ected lymph node group

as a result of compression of surrounding tissues.

Aim. To analyze clinical manifestations and treatment effi cacy in pediatric Catleman’s disease.

Materials and methods. We conducted a retrospective analysis of 14 patients with histologically confi rmed Castleman’s disease. The age of patients varied from 5 months to 16 years old.

Results. The age of onset varied from neonatal period to 16 years old. Nine of the studied patients had unicentric form of disease, while only 2 had multicentric form and one patient

had two groups of lymph nodes aff ected. The majority of the patients (9 patients) had hyaline-vascular variant of the disease, one patient had plasma-cell type and two patients

had the mixed pathomorphological type. The reason for seeking medical attention in three patients was visible unilateral enlargement of the neck group lymph nodes. Four patients

initially presented such symptoms as anemia, fatigue, faintness, low-grade fever and elevation of CRP and ESR. Two patients had underlying primary immunodefi ciency – Wiskott–

Aldrich syndrome and developed systemic lymph node hyperplasia. The biopsy and further histological study showed plasma cell type and mixed type of the disease in those patients,

respectively. Four patients were diagnosed after undergoing X-ray studies for infections (three) and osteomyelitis (one).

The aff ected lymph nodes were completely excised in 8 patients with unicentric form and there were no signs of relapse of the disease in the follow-up. Two patients have

undergone bone marrow transplantation as a treatment of the main disease (2 patients with Wiskott–Aldrich syndrome, of which 1 patient showed long-term remission of the

Castleman’s disease in the follow-up and 1 patient is still in-patient, receiving treatment in our center). One patient with multicentric form received several courses of anti-cytokine

and immunosuppressive treatment including tocilizumab, that was further enhanced by cyclophosphamide, vincristine and prednisolone (total of 5 blocks), rituximab (6 infusions),

bortezomib with bendamustine (total of 6 blocks) with positive eff ect. However the disease relapsed, so we decided to initiate treatment with JAK1 and JAK2 tyrosine kinase

inhibitor – ruxolitinib.

Conclusion. Castleman’s disease is a multifaceted disorder, as it can be treated easily in some cases and in other cases present therapeutic challenge to a physician.

A B S T R A C T N O . : O P - 4 8 2

Lymphomas in primary immunodefi ciency patients: single center experience

E.V. Deripapa, О.А. Shvets, D.S. Аbramov, N.V. Miakova, А.Yu. Shcherbina

Federal Research and Clinical Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Introduction. Primary immunodefi ciencies (PID) comprise a diverse group of disorders with underlying genetic defects of immune system. Besides severe, life-threatening infections

patients with PID have higher frequency of autoimmune disorders and tumors, mostly of lymphoreticular origin, lymphomas being the most frequent malignancy (from 5 to 50 %

in various PID groups).

Aim. To analyze lymphoma frequency in PID patients followed in our Center.

Materials and methods. Case histories of 280 PID patients ages 2 month – 21 years followed in our Center between years 2012 and 2015 were retrospectively analyzed.

PID diagnosis was made based on ESID criteria and in most cases confi rmed genetically. Lymphoma diagnosis was made based on patomorphology, and graded based on computer

tomography visualization fi ndings.

Results. 27 patients (7 females and 20 males) out of 280 PID patients developed lymphomas (9.6 %). Median age of lymphoma onset was 8.8 years.

The group with highest frequency of lymphomas consisted of patients with DNA repair defects (ataxia-telangiectasia, Nijmegen syndrome) 15 patients out of 37 patients (40 %).

Seven patients with various combined immunodefi ciencies developed lymphomas. There were single cases of lymphoma in patients with Wiskott–Aldrich syndrome, cartilage hair

hypoplasia syndrome, X-linked lymphoprolifereative disorder and PI3Kd defect.

Large B cell lymphoma occurred in 44 % cases, followed by Burkett (22 %) and Hodgkin’s (19 %) lymphomas. Interestingly, in 8 out of 15 patients with DNA-repair defects diagnosis

of PID was made only after lymphoma diagnosis.

Conclusion. Our study demonstrates that the overall frequency of lymphoma occurrence in PID patients is quite high – almost 10 %, and in the group of DNA-repair defects it reached

40 %. Early diagnosis of PID and oncological awareness is important for monitoring of these patients.

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A B S T R A C T N O . : O P - 4 8 3

Certain ELANE mutations predispose to more severe course

of severe congenital neutropenia

E.A. Diordiaeva, T.V. Varlamova, E.V. Raikina, A.A. Maschan, A.Yu. Shcherbina

Federal Research and Clinical Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

ELANE mutations are the most common cause of severe congenital neutropenia (SCN). Mutations occur in all exons of the gene, in most introns and 5’UTR. So far no signifi cant

correlation of the type and location of the mutation and severity of the disease has been found. Yet, V. Makaryan et al. reported, that several frequently occurred mutation

(e.g. at the positions C151 and G214) lead to more severe phenotype as judged by poor response to G-CSF treatment and high frequency of MDS and AML development

(Abstract 3275, ASH Meeting 2012).

In confi rmation of this observation, we report here four patients with SCN harbouring ELANE mutations. The patient with ELANE mutation at the position C151 had a very poor response

to high dose of G-CSF – upon treatment with up to 50 ug/kg/day of G-CSF maximal neutrophil counts achieved were only 500 cells/ul. Moreover, this patient developed MDS very early –

at the age of 13 months. Second patient with the ELANE mutation at the position C151 also is a very poor responder to G-CSF. He is 20 months old and has no signs of AML/MDS yet.

Therefore, we strongly suggest that at least these two mutations in ELANE should be considered unfavorable in terms of prognosis and patients should be considered for early HSCT.

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A B S T R A C T N O . : O - 1 9 5

Functional activity of platelets and spatial thrombin generation after platelet concentrate

transfusion in children after hematopoietic stem cell transplantation

E. Koltsova, A. Balandina, I. Demina, S. Radygina, F. Ataullakhanov, D. Balashov, M. Panteleev

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: platelet transfusion, hematopoietic stem cell transplantation, haemostasis, thrombin

Introduction. Allogenic platelet transfusions are important in clinical practice as an effi cient treatment of persistent thrombocytopenia, which is a common complication after

hematopoietic stem cell transplantation (HSCT).

Aim. The aims of this study are to investigate eff ects of platelet transfusion on platelet functions, spatial clot growth and thrombin generation in children after HSCT.

Materials and methods. Ten patients (1–17 y. o.) were investigated before and 1 hour after platelet transfusion. Eight out of ten patients were given 100 ME/kg/h heparin.

Concentrate was prepared by apheresis, γ-irradiated (25 Gy), leukofi ltrated and stored for 3–5 days before the transfusion. Mean concentrate dose was 6.5 ± 5.4 ml/kg. Platelet count

was performed in whole blood and platelet rich plasma (PRP) before and after the transfusion. Platelet α-granules and dense granules secretion markers (CD62P and Mepacrine),

non-activated and activated form of the glycoprotein GPIIb/IIIa (CD61 and PAC-1), glycoprotein GPI (CD42b) and PS (annexin V) expression were measured by fl ow cytometry.

The integral estimation of haemostasis state was performed with pre-production model of Thrombodynamics-4D Analyser System (HemaCore Labs). Coagulation was activated

by immobilized tissue factor (TF) in PRP. Spatial clot growth was registered by light scattering. Thrombin generation in space and time was determined using the registration

of the UV-LED excited fl uorescence of thrombin-activated 7-amino-4-methylcoumarin (AMC) substrate.

Results. Platelet count in PRP showed signifi cant eff ect of transfusion (means±SD before/after the transfusion: 8.0 ± 6.6/52.3 ± 25.2 × 10^3/μl, p less then 0.001). However,

the platelet functional activity assay showed reduced platelet activity per single platelet both before and after the transfusion: secretion of dense granules (Mepacrine index

1.37 ± 0.44/1.35 ± 0.19, reference interval 2.3–3.4), α-granules (CD62P 6713 ± 2388/5968 ± 2159 a.u., reference interval 9750–12 300 a.u.) and expression of non-activated

GPIIb/IIIa (CD61 3179 ± 1468/3290 ± 715 a.u., reference interval 4000–7800 a.u.) stayed depressed after transfusion and did not change signifi cantly. PS expression was normal

before and after the transfusion (Annexin V 0.4–14.0/4.3–40.0 %, reference interval 0,8–38,1 %). The estimation of haemostasis state with Thrombodynamics-4D assay showed the

change in spatio-temporal distribution of thrombin. It changed from diff use to front-shaped after the transfusion, though did not reach wave-shaped distribution with distinguishable

peaks, observed in plasma of healthy volunteers. The assay revealed a signifi cant increase of clot growth rate V (25.4 ± 7.7/32.5 ± 3.9 μm/min, P = 0.004, reference interval

34–40 μm/min) and produced amount of AMC (122 ± 48/207 ± 35 μM*mm, p less then 0.001, reference interval 180–320 μM*mm), associated with amount of thrombin.

Conclusion. The functional activity of platelets is reduced in patients after HSCT and stays reduced after the platelet transfusion. However, due to the signifi cant increase in platelet

count, the transfusion integrally leads to normalization of clotting, although the clot growth and thrombin propagation parameters do not reach such of healthy volunteers.

The study was supported by the RFBR grants 15-34-70014, 15-54-45036 and 14-04-00670.

HEMATOPOIETIC STEM CELL TRANSPLANTATION

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A B S T R A C T N O . : P - 1 9 8

Experience of treatment of chronic graft-versus-host disease using

the method of extracorporeal photopheresis

L. Hushchina, N. Kirsanova, G. Mareika, N. Minakovskaya, D. Prudnikov, A. Alexeychik

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: chronic graft-versus-host disease, alloHSCT, ECP

Introduction. Chronic graft-versus-host disease (cGVHD) is the most serious and common complication of alloHSCT, it occurs in 30 % to 60 % of patients. cGVHD represents the major

cause of non-relapse mortality and late morbidity in transplant survivors.

Aim. The aim of our study was to assess the effi cacy of extracorporeal photopheresis (ECP) in cGVHD.

Materials and methods. Patients and methods: 9 pts (5 female, 4 male), age from 2 to 24 y.o. (median 11 y.o.) suff ering from ALL – 3 pts, AML – 2 pts, ABL – 1 pt, CML – 1 pt,

thalassemia – 1pt, chronic granulomatous disease – 1 pt. All pts received allo-HSCT: matched unrelated HSCT – 2 pts (22 %), mismatched unrelated HSCT – 1 pt (11 %), matched

related HSCT – 5 pts (56 %), haploHSCT – 1 pt (11 %). Onset type of cGVHD is progressive (persistent signs and symptoms of aGVHD) – 7 pts (78 %), quiescent (no clinical signs or

symptoms of aGVHD) – 1 pt (11 %), de novo (no prior history aGVHD) – 1 pt (11 %). Frequency of organ involvement: skin – 9 pts (100 %), joints – 1 pt (11 %), liver – 2 pts (22 %),

oral mucosa– 4 pts (44 %), gastrointestinal – 1 pt (11 %), genitalia – 1 pt (11 %), eyes – 1 pt (11 %), lung – 1 pt (11 %). ECP were used as second or third line therapy. Fourpatients

received only ECPas immunosupressivetherapy, 5 pts continued to receive steroid and ECP. Corticosteroid status is dependent – 1 pt from 5, refractory – 4/5 pts. Patients received ECP

twice weekly during fi rst 2 weeks, then 1 times per week during 1 month and after once monthly. Patients received 2 to 21 cycles of ECP (median 10 cycles). Median time of carrying

out ECP is 12.8 month (from 4.1 to 87.2 month) from HSCT date.

Results. Complete resolution (CR) was achieved in 2 pts (22 %), partial resolution (PR) – in 6 pts (67 %); absence of response was in 1 pt (11 %). Steroid therapy could be discontinued

in 4 from 5 pts (80 %) (1 pt with CR, 3 pts with PR), decrease in steroid dosage with PR of GVHD clinical signs – 1 pt (20 %). No patients had sideeff ects during or immediately after

the procedure ECP.

Conclusion. Our results indicate that ECP is eff ective method for treatment of chronic GVHD and allows completely withdrawal steroid therapy in most patients.

A B S T R A C T N O . : P - 2 0 0

The acute form of “graft versus host diseases” in children: prognostic factors

and treatment of mesenchymal stem cells steroidrefractory form

N. Minakovskaya

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: graft versus host diseases, allogeneic hematopoietic stem cell transplantation, mesenchymal stem cells

Introduction. Steroidrefractory forms of acute “graft versus host diseases” in children after allogeneic hematopoietic stem cell transplantation oneof the main causes of therapy-

related death.

Aim. Is to improve the treatment steroidrefractory forms of acute “graft versus host diseases” in children with hematologic malignancies and primary immunodefi ciency after

allogeneic hematopoietic stem cell transplantation by detection of risk factors and transplantation of mesenchymal stem cells.

Materials and methods. The study included 218 SCT to 214 children (age 6 months – 18 years, mediana 10.5 y.o.) in the period from 1998 to 2014 . The largest number of SCT

performed to children with acute leukemia (ALL – 72 (33 %), AML – 41 (18.8 %)) and SAA – 45 (20.6 %). 20 patients with steroidrefractory aGvHD for treatment was carried

mesenchymal stem cells (MSC) and 11 patients without MSC constituted the control group. All patients and control groups were matched for age and sex.

Results. The incidence of acute GVHD in children after SCT found in 120 (55 %) cases of 218 transplants. Factors aff ecting the development of life-threatening III–IV stages

steroidrefractory acute GVHD are incompatibility antigens HLA-system – 13 (65 %, P = 0.035), donor age older than 30 years – 17 (65.4 %, P = 0.0268) and unrelated allogeneic HLA-

incompatible HSCT – 11 (78.5 %, p=0.0266). Number of TNC in the graft a 5.0 × 108/kg body weight of the recipient is a risk factor for stage IV steroidrefractory acute GVHD – 9 (32.1 %).

Conclusion. Carrying allogeneic transplantation of MSC led to a complete and partial remission of steroidrefractory acute GVHD in 16 (80 %) of the 20 patients, in the control group

partial remission occurred in 3 (27 %) patients.

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A B S T R A C T N O . : O - 2 2 5

Survival and treatment of children with acute graft versus host disease:

an 8-year single-сentre experience

A. Kozlov, T. Bykova, Yu. Fedukova, M. Kucher, E. Morozova, E. Semenova, A. Borovkova, K. Ekushov, S. Bondarenko,

O. Slesarchuk, I. Kulagina, E. Babenko, A. Alyansky, M. Estrina, L. Zubarovskaya, B. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology,Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia

Key words: acute graft versus host disease, methylprednisolone, steroid-refractory, extracorporeal photopheresis, anticytokine therapy

Introduction. Despite remarkable progress in the fi eld of allogeneic hematopoietic stem cell transplantation (allo-HSCT) the rate of transplant-related mortality remains high due

to severe transplant-associated complications and acute graft host disease (aGVHD) is among the most frequent and life-threatening.

Aim. Evaluation of the overall survival (OS) in children with aGVHD that were treated with methylprednisolone (MEP) as fi rst-line therapy and extracorporeal photopheresis,

anticytokine therapy or alemtuzumab as second line therapy.

Materials and methods. A total of 97 children (range 1–18 years) with aGVHD grade II-IV after allo-HSCT were included in the study. In all cases the fi rst line therapy consisted

of 1–2 mg/kg of methylprednisolone (MEP). Response was evaluated 3 days after therapy initiation. If there was no response after 3 days of steroids or if aGVHD fl ared during

the corticosteroid taper, then steroid-refractory aGVHD was diagnosed. Extracorporeal photopheresis (ECP) was used as further therapy in 31 (48 %) patients, anticytokine therapy

was used in 29 children (45.5 %) (etanercept (n = 12), infl iximab (n = 9), daclizumab (n = 8)) and alemtuzumab in 4 (6.5 %). Both groups (responders and non-responders to

MEP) were homogenous in terms of such characteristics as median age (8 years vs 8.5 years), sex (female 41 % vs 39 %), diagnosis (acute leukemia 73 % vs. 69 %), donor type

(unrelated donors 75 % vs 67 %, P = 0.8), transplant source (bone marrow 67 % vs 63 %) and conditioning regimen (myeloablative 36 % vs 45 %, P = 0.2), respectively. Nevertheless,

these groups (responders and non-responders to MEP) were heterogeneous in terms of aGVHD stage (severe 24 % vs 59 %, P = 0.03), respectively. Overall survival was estimated by

the means of Kaplan–Meier analysis and comparison of two survival curves was done by the means of log-rank test.

Results. Five-year overall survival (OS) of patients included in the study was 48.6 %. Median follow-up was 20.5 moths (range 6–124). Complete response to MEP was registered

in 33 (34 %) patients and inadequate response in 64 (66 %) patients. Complete responders didn’t require any further immunosuppression and MEP could be successfully discontinued,

meanwhile non-responders required additional immunosuppressive treatment options. Patients with steroid-sensitive course of aGVHD demonstrated 5-year OS of 69 % and children

with steroid-refractory aGVHD showed 5-year OS of 39 % (P = 0.08). Overall response to the second-line therapy was 68 % after ECP (complete response – 32 %, partial response – 36 %)

and 70 % after anticytokine therapy (complete response – 42 %, partial response – 28 %), P = 0.77. Among 4 patients with alemtuzumab 2 complete responses and 1 partial response

were registered. Children that responded to second-line therapy of aGVHD demonstrated 5-year OS of 54 % which didn’t statistically diff er from OS of steroid-sensitive group (P = 0.33).

Conclusion. Prognosis of children with steroid-refractory aGVHD still is less favorable than in steroid-sensitive group despite administration of such modern treatment options as ECP

and anticytokine therapy. Prognosis of children that respond to second line therapy of aGVHD is comparable to prognosis in steroid-sensitive aGVHD.

A B S T R A C T N O . : O - 2 2 7

Invasive fungal diseases in adolescents and young adults after allogeneic hematopoietic

stem cell transplantation

M. Popova1, A. Volkova1, O. Ayzsilnieks1, O. Pinegina1, S. Ignatyeva2, K. Ekushov1, O. Slesarchuk1,

M. Vladovskaya1, L. Zubarovskaya1, N. Klimko2, B. Afanasyev1

1First Pavlov State Medical University of St. Petersburg, Russia;2North-Western State Medical University named after I.I. Mechnikov, Saint-Petersburg, Russia

Key words: IFD, allo-HSCT, adolescents and young adults, epidemiology, IA

Introduction. Invasive fungal diseases (IFD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) are still actual problem despite improving prognosis recently.

Data of IFD in adolescents and young adults after allo-HSCT are limited.

Aim. This study focuses on epidemiology of IFD in adolescents and young adults after allo-HSCT.

Materials and methods. In retrospective single-center study from Jan 2013 to Dec 2014 were included 80 pts after fi rst allo-HSCT. The median age was 20 y.o.(15–25). Underlying

diseases were acute leukemia – 71.5 %, lymphoma – 15 %, not malignant diseases – 7.5 %, other – 6 %. Allo-HSCT from MUD was performed in 67 %, MRD – 24 %, haplo – 9 %.

RIC were used in 72.5 %. EORTC/MSG 2008 criteria for IFD diagnosis were used, probable and proven IFD were taken into analysis. Active diagnostic strategy: bronchoscopy with BAL in

pts with CT-scan lung lesions before HSCT was used. “Active IFD” means IFD diagnosed just before allo-HSCT. IFD-events mean new IFD and relapse of IFD in pts who had it before allo-HSCT.

Results. IFD was diagnosed before allo-HSCT in 19 % pts: invasive aspergillosis (IA) – 11 pts, hepatosplenic candidiasis (HSC) – 3 pts, and one patient had two IFD (IA+HSC).

Complete response to antifungal therapy was in 40 % pts, partial response – 26,7 %, and “active IFD” – 33,3 %. Secondary antifungal prophylaxis was made with voriconazole (80 %).

Pts without IFD before allo-HSCT (n = 65) received primary prophylaxis with fl uconazole – 85 %. Cumulative incidence of IFD-events at 2 years after allo-HSCT was 19 %: 14 cases of

new IFD and one relapse of IA. Median time to IFD-event onset was D+43 (14–577). Incidence of IA was 15% with median time to onset D+27.5 (14–577), IC – 2.5 % with median

time to onset D+226.5 (54–399), pneumocystis pneumonia (PCP) – 1.25 %, day of onset – 43. The main sites of infection were lungs – 86.7 %. Risk factors for IFD after allo-HSCT in

pts without IFD before HSCT were ageless 18 y.o. (P = 0.03), not malignant underlying disease (P = 0.003), active underlying disease at the moment of HSCT (P = 0.01), neutropenia

(grade 4) more 20 days (P = 0.004), and acute GvHD (P = 0.008). Seven pts (58.3 %) with IA were treated with voriconazole as a fi rst line treatment, all pts with IC – ehcinocandins,

and patient with PCP was treated with trimethoprim-sulfamethoxazole. 12-weeks overall survival (OS) from day of IFD diagnosis after allo-HSCT was 93.3 %. 12-weeks OS in pts with

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IA was – 91.7 %, two pts with IC and one patient with PCP were alive at 12 weeks after IFD diagnosed. Voriconazole as a fi rst line treatment improved 12-weeks OS in pts with IA after

allo-HSCT (100 % vs 80 %, P = 0.2). 100-days OS after allo-HSCT was 85 %, 2-year OS after allo-HSCT was 67.5 %. There was no statistical diff erence in 2-year OS in pts with (53.3 %)

or without IFD (70.8 %) before allo-HSCT (P = 0.3).

Conclusion. Incidence of IFD in adolescents and young adults before allo-HSCT was 19 %. Cumulative incidence of IFD at 2 years after allo-HSCT was 19 %. The main IFD was IA – 80 %.

Risk factors for IFD after allo-HSCT were ageless 18 y.o., not malignant underlying disease, active underlying disease at the moment of HSCT, neutropenia (grade 4) more 20 days,

and acute GvHD. 12-weeks OS from IFD diagnosis after allo-HSCT was 93.3 %. IFD before and after allo-HSCT did not impair the outcome of the transplantation in adolescents and

young adults with eff ective diagnosis, treatment and secondary prophylaxis have been used.

A B S T R A C T N O . : P - 2 5 2

New HLA-haplotypes as the result of crossover

V. Zakharova, O. Shragina

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: HLA, MHC, crossover, double crossover, PCR, SSP, sequence based typing

Introduction. Of all the genetic and non genetic factors that can infl uence stem cell transplant outcome, the most important is HLA matching. A most striking feature of the HLA

system besides its many closely linked, functionally related and highly polymorphic genes is the signifi cant over- and underrepresentation of certain allelic combinations, called

linkage disequilibrium.

In rare cases, the HLA genes can be separated by genetic recombination (i.e. crossover of genetic material between homologous chromosomes during meiotic division).

This recombination occurs quite rarely as established from testing large numbers of famylies. Recombination is evident when family typing studies are performed and greatly

complicate the search for a marrow donor.

Aim. HLA-typing for searching of related donor for hematopoetic stem cell transplantation.

Materials and methods. One patient with acute myeloid leukemia (AML) and one patient with unknown diagnosis and family members (two parents and one sibling in each family)

were included in the study.

HLA-typing low and high resolution for loci HLA-A*, B*, C*, DRB1*, DQB1* was performed by PCR-SSP (Sequence-specifi c primer, Invitrogen, USA) and SBT (Sequence-based typing,

Invitrogen, USA).

Results. HLA typing showed double crossover in family 1 and single crossover in family 2. Father’s haplotypes in patients and siblings are not shown.

Family 1:[patient: A*03, C*06, B*57, DRB1*04, DQB1*03:01];

[sibling: A*24, C*06, B*57, DRB1*07, DQB1*03:03];

[mother: A*24, C*06, B*57, DRB1*07, DQB1*03:03 / A*03, C*07, B*07, DRB1*04, DQB1*03:01].

Family 2:[patient: A*03, C*04, B*35, DRB1*01, DQB1*05];

[sibling: A*03, C*04, B*35, DRB1*15, DQB1*06];

[mother: A*03, C*04, B*35, DRB1*01, DQB1*05 / A*01, C*07, B*07, DRB1*15, DQB1*06].

Case in family 1 shows a double recombination between the HLA-A/HLA-C and HLA-B/HLA-DRB1 loci. Case in family 2 illustrate a recombination between the HLA-B and HLA-DRB1 locus.

Based on linkage disequilibrium and haplotype frequencies it is likely that recombinant haplotype is present in proband rather then sibling in family 1 and present in sibling rather

then proband in family 2.

Conclusion. Althgouh crossover in HLA genes is a rare event, this cases illustrate that such recombination in hot spots resulted in mismatch between the patient and potential donor.

Even if siblings are matching it is also useful to determine the HLA haplotypes of the parents if possible. In cases similar to described above grandparents, cousins, aunts and uncles

should be typed to get exact distribution of haplotypes.

A B S T R A C T N O . : P - 2 5 7

The safety of hematopoietic stem cell apheresis in children with oncological diseases

with body weight up to 15 kg

I.B. Kumukova, P.E. Trakhtman, E.Ye. Kurnikova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: hematopoietic stem cell apheresis

Introduction. Hematopoietic stem cell (HSC) apheresis in children with low body weight is associated with a number of technical diffi culties and safety problems: vascular access,

a risk of hypervolemia and hypothermia, the emerging necessity of large volume leukapheresis (LVL), a risk of citrate toxicity and its underestimation, patients’ sedation in some cases.

Aim. The analysis of the experience of HSC apheresis in children with oncological diseases and body weight up to 15 kg.

Materials and methods. Overall, 77 HSC aphereses were performed in 76 patients. The analysis did not include 1 repeat apheresis. The median age comprised 33.7 (6–84) months,

the median body weight – 12.6 (6–15.8) kg. The nosological structure: neuroblastoma (n = 66), medulloblastoma (n = 4), nephroblastoma (n = 2), Ewing sarcoma (n = 2), HEM

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yolk sac tumor (n = 1), pleuropulmonary blastoma (n = 1). HSC mobilization – granulocyte colony-stimulating factor 10–20 mg/kg/d for 3–7 days with the use of plerixafor

if needed. The HSC apheresis procedure was conducted using COBE Spectra system (n = 9) or Spectra Optia (n = 68), with the preliminary fi lling of erythrocyte-containing blood

components with donor erythrocyte suspension in 68 cases out of 76. HSC aphereses in 5 children less than 10 months old with body weight less than 9 kg were carried out at intensive

care unit under constant monitoring and sedation and with prophylactic administration of calcium gluconate. Other procedures were performed at Transfusion Department without

intensive monitoring and calcium gluconate administration.

Results. Only 1 patient out of 76 needed a repeat apheresis to achieve optimal CD34+ levels. In 8 patients, HSC apheresis was performed via the previously implanted two-channel

central venous catheter (CVC); 8 patients had a replacement of a one-channel subclavian CVC by a two-channel one (Hickman 7F or Certofi x 5F). 60 patients needed an implantation

of a temporary CVC with femoral access. 11 patients out of these 60 had mild catheterization complications such as repeat venepunctures or haemotomas at puncture sites.

In 1 case, the internal jugular vein was damaged during a subclavian vein catheterization attempt wherefore apheresis was postponed for 1 month. The median apheresis duration was

289 (156–444) min. The median circulating blood volume processed per a procedure was 2.85 (1.5–5.2). The characteristics of apheresis products: the median NC/kg– 12 × 158/kg

(2.29 × 108/kg – 30.14 × 108/kg), the median CD34+ – 13.83 × 106/kg (2.37 × 106/kg – 63.37 × 106/kg). In 35 cases out of 76, LVL was carried out, and only in 1 case there was

a mild citrate reaction. In 8 patients older than 4 years of age with body weight ranging from 14.5 to 15.8 kg and hemoglobin levels higher than 115 g/l, contour fi lling with erythrocyte

suspension was not performed. There were no complications associated with anaemia, hupovolaemia, transfusions. The median thrombocyte level after apheresis comprised

109 × 109/l (38–311 × 109/l). A decrease in hematocrit levels after apheresis was registered in 4 children.

Conclusion. HSC apheresis in younger children requires additional eff orts and particular attention as well as a high level of competence of the personnel. In case of an adequate

preparation for apheresis, the procedure seems to be quite safe and, in the majority of cases, does not require intensive monitoring and intervention. There were no grave complications

of apheresis registered. Thrombocytopenia and a decrease in hemoglobin levels after apheresis were not threatening. LVL does not increase the risk of complications as compared with

the standard HSC apheresis. According to the results of our study, the majority of complications in young children are associated with the need for an adequate vascular access - the

implantation of an additional CVC.

A B S T R A C T N O . : O P - 2 5 8

The role of bronchoscopy in the diagnosis of invasive pulmonary mycosis

in children after allogeneic hematopoietic stem cells transplantation

A. Volkova1, M. Popova1, K. Ekushov1, T. Bogomolova2, S. Ignatyeva2, B. Smirnov3,

L. Zubarovskaya1, B. Afanasyev1, N. Klimko2

1First Pavlov State Medical University of St. Petersburg, Russia; 2North-Western State Medical University named after I.I. Mechnikov, Saint-Petersburg, Russia; 3Saint-Petersburg Electrotechnical University “LETI”, Russia

Key words: invasive pulmonary mycoses, bronchoscopy

Introduction. Early diagnosis of invasive pulmonary mycoses (IPM) in patients after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is one of the factors positively

infl uencing the survival.

Aim. The role of bronchoscopy in early diagnosis of IPM in children after HSCT is not clear.

Materials and methods. In prospective single-center study in 2009 to 2014 yy we evaluated the safety and effi cacy of bronchoscopy (n = 209) with bronchoalveolar lavage (BAL)

for the diagnosis of IPM in 135 children after allo-HSCT. The median of age was 12 (from 1 to 18) years old. In a specialized endoscopy room or ICU (13 %) bronchoscopy was made

with EVIS EXERA II Olympus endoscopic video system with an external diameter of the distal end tubes of the 3.6 mm and 4.9 mm. Diff erent methods of anesthesia were performed,

depending on the age and the degree of respiratory failure of patients with mandatory monitoring of vital functions and oxygen levels. The obtained samples were sent to the lab

for microscopy with white calcofl uor, culture, and galactomannan (GM) test (Platelia Asregillus, Bio-Rad). In 4 (3 %) patients there were signs of endobronchial fungal growth, and

we performed biopsy during bronchoscopy. Two (1.5 %) patients with negative BAL underwent percutaneous automatic biopsy and lung lobectomy for diagnosis of IPM. EORTC/MSG

2008 criteria for IFD diagnosis were used, only “probable” and “proven” IFD were taken into analysis.

Results. No major complications occurred during bronchoscopy. IPM was found in 41 % patients with lesions on CT scan. 89 % IPM were “probable” and 11 % “proven” according to

EORTC/MCG 2008 criteria. The results of GM tests in BAL fl uid were positive in 66 % samples. Culture and microscopy were positive in 43 % cases. Main pathogens were Aspergillus

spp. (73 %) and mucormycetes (14 %). Rare pathogens were Acremonium spp., Paecilomyces spp., Fusarium spp., Alternaria spp. and P.jirovecii. Two or more pathogens were detected

in 9 %. In multivariate analysis, galactomannan assay in BAL fl uid showed a higher sensitivity compared to microscopy and culture (83.3 % and 46.3 %, respectively).

Conclusion. In children after allo-HSCT bronchoscopy with modern video endoscopy equipment and adequate anesthetic is eff ective and safe method of diagnosis of invasive

pulmonary mycoses.

Detection of galactomannan in BAL is more eff ective than microscopy and culture for diagnosis of invasive aspergillosis in in children after HSCT.

The most diagnostic effi ciency is achieved with all three methods – GM test in BAL, microscopy and culture with.

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A B S T R A C T N O . : O P - 2 6 1

The related factors of infl uencing unrelated umbilical blood transplantation

on children with Wiskott–Aldrich syndrome

Xin-ni Bian, Pei-fang Xiao, Jun, Shaoyan Hu

Children’s Hospital of Soochow University

Key words: Wiskott–Aldrich syndrome (WAS), unrelated cord blood transplantation (UCBT), granulocyte engraftment, platelet engraftment, HLA high resolution, nutritional status

Introduction. More and more infants with Wiskott–Aldrich syndrome (WAS) have been recently diagnosed with WAS gene sequencing available in China. It has been accepted that

hematologic stem cell transplantation (HSCT) is one of the most curable treatment. A few institutions preferred well matched unrelated peripheral blood stem cell transplantation

(PBSCT) on WAS patients in China. However, patients with WAS faced two problems: one is well matched unrelated donor is diffi cult to fi nd in the Chinese bone marrow bank, another

is unrelated donor refuse donate stem cells after selected as a donor. As a result, many infants have to wait for suitable donor at the risk of increasing bleeding, serious infection, even

death. So unrelated cord blood is a perfect option for such patients.

Aim. To explore the outcome unrelated donor cord blood transplantation (UCBT) on children with Wiskott-Aldrich syndrome (WAS) and impacting factors, such as HLA high resolution,

nutritional status, virus infection.

Materials and methods. From February to September of 2015 fi ve patients diagnosed as WAS were treated with UCBT in our center. The factors as the nutritional status prior to

transplantation, age of transplantation, HLA matched degree, post-transplantation graft versus host disease (GVHD), infection status were analyzed. Follow-up lasted to January 2016.

Conditional regimen include Busulfan at 1.0 mg/kg.q6h for 4 days, Fludarabine 40 mg/m2.d for 4 days, Cyclophosphamide 60 mg/kg.d for 2 days and ATG 2.5 mg/kg.d for 3 days. GVHD

prevention consists of MMF at 20 mg/kg.d and Cyclosporine at 3 mg/kg.d.

Results. According to the STR results, fi ve children with WAS were cured with UCBT. Granulocyte engraftment was 15 days, 12 days, 11 days,14 days and 15 days, respectively.

Platelet engraftment was 39 days, 16 days, 17 days, 34 days and 36 days, respectively. HLA high resolution matched between receipt and donor was 8/10, 10/10, 5/10, 10/10, 10/10,

respectively. Three of the patients suff ering CMV viremia before transplantation showed a platelet engraftment delay. One case with severe malnutrition showed severe GVHD of skin

and intestine and recurrentsevere infections after transplantation. Follow-up 4 months to 12 months, 5 cases keep a stable value of STR at 100 %.

Conclusion. CMV viremia and nutritional status before transplantation infl uenced the platelet engraftment and complications of UCBT. The degree of HLA high resolution has no

relations with the engraftment of neutrophil and platelet and complications of UCBT.

A B S T R A C T N O . : P P - 2 7 5

Second haploidentical stem cell transplantation as a salvage therapy for children

with acute leukemia relapsed after fi rst allo-HSCT

P. Kozhokar, O. Paina, A. Borovkova, E. Semenova, A. Rats, S. Razumova, K. Ekushov, Yu. Fedukova,

O. Slesarchuk, L. Zubarovskaya, B. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation,First Pavlov State Medical University of St. Petersburg, Russia

Key words: acute leukemia, allogeneic HSCT in children, relapse, second haploidentical HSCT

Introduction. Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for acute leukemia. However post-HSCT relapse remains an important

cause of treatment failure with relapse rates ranging from 30 % to 70 %. Optimal treatment of relapse after HSCT remains unclear.One of possible options might be second

transplantation from another donor.

Aim. The aim of study was to evaluate the effi ciency of second haploidentical HSCT from haploidentical donor for treatment of relapse of acute leukemia after fi rst allogeneic HSCT

in children

Materials and methods. We retrospectively analyzed outcomes of 21 second haploidentical HSCT performed in our Institute for children with acute leukemia (AML – 6, ALL – 14,

mixed lineage leukemia-1), relapsed after the fi rst HSCT. Median age at the time of fi rst HSCT – 5 y.o. (13 m.o. – 17 y.o.), median age at second HSCT – 6 y.o. (22 m.o. – 17 y.o.).

Donor for the fi rst HSCT were matched unrelated (n = 7), matched related (n = 6), haploidentical (n = 6), singeneic (n = 1). The median time from fi rst to second allo-HSCT – 9 months

(34 days – 28 months). In case of fi rst haploidentical donor second HSCT were from another donors. Cytoreductive therapy prior to second HSCT was performed in 19 pts. There was no

response in 16 pts, 1 pt achieved complete remission and 4 pts were transplanted in aplasia. The conditioning regimen for second HSCT was myeloablative in 4 pts (conventional – 2,

reduced toxicity – 2), reduced intensity – 17 pts.

Results. 19/21 patients achieved engraftment, median of neutrophyl engraftment – 20 days (12–34 days). At the time of analysis 10 pts are alive with median follow up of 5 months

(2 months – 88 months).Five – years overall survival (OS) – 42.5 %. The period between the fi rst to second HSCT had no statistically eff ect on OS. Relapse rate after second allo-HSCT

was 42 %. Non relapse mortality – 21 %.The causes of death were relapse or progression of disease (7 pts), acute GvHD (3 pts), infectious complications (1 pt).

Conclusion. Our data showed that the second haplo-HSCT is acceptable choice for treatment children with acute leukemia relapsed after fi rst allo-HSCT. Further trials are needed to

defi ne the role of second haplo – HSCT including combination with additional treatment after transplantation.

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A B S T R A C T N O . : O - 2 7 9

Cytomegalovirus retinitis in hematopoetic stem cell pediatric transplant patients

B.S. Pershin, O.A. Boginskaya, A.B. Smirnova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: cytomegalovirus retinitis

Introduction. Cytomegalovirus (CMV) retinitis is associated with immunosupression and can cause irreversible vision loss

Aim. A clinical case of a patient 5 y.o. with Wiskott–Aldrich syndrome.

Materials and methods. Stem cell transplantantion (SCT) was performed from an unrelated donor transplant with processing TCRåß/CD19-deletion. Ocular examination before SCT

didn’t reveal any pathology, visual acuity (VA) was 20/20.

Results. On 11 day after SCT CMV viremia was 1386 copies/ml and decreased on 14 day to 500 copies/ml. The negative results confi rming CMV absence were achieved on 16 day after

BMT under essential supplement of intravenous Ganciclovir.

Ophthalmic exam on 45 day after SCT revealed a single white crescent formed focus with blurring edges in macular area, measured as 1.5 of optic disc diameter. VA of the left eye

deteriorated to 20/63. Three intravitreal injections of Ganciclovir 2 mg/0.1 ml were performed once a week.

With each implemented procedure the described focus became more transparent. After third injection it was absolutely eliminated. Previously involved retina appeared

to be unaff ected with exceptionally thin line of retinal pigment epithelium dispersion on the periphery of macular area. VA improved to 20/20

Conclusion. Due to the diffi culties in defi ning VA in infants and toddlers it is important to remember that CMV retinitis may be asymptomatic. Regular ophthalmological monitoring

in children after SCT is crucial. Early performance of intravitreal injections of specifi c antiviral agents allows to prevent spreading of infl ammatory process in retina and maintain visual

functions.

A B S T R A C T N O . : O P - 2 8 5

The role of bone marrow stromal cells functional characteristics

in post-transplant hematopoietic reconstitution

N. Tsvetkov, I. Barkhatov, A. Shakirova, D. Romaniuk, A. Potter, L. Zubarovskaya, B. Afanasyev

First Pavlov State Medical University of St. Petersburg, Russia

Key words: hematopoietic stem cell transplantation, bone marrow stromal cells, hematopoietic reconstitution

Introduction. The role of the stromal microenvironment in hematopoietic stem cell transplantation (HSCT) is based on nonlineage-specifi c eff ects on proliferation and diff erentiation

of HSCs. Defi cient graft functioning observed in some cases necessitates development of functional tests for the stromal cells, in order to provide clinical indications for co-transplanting

of hematopoietic and bone marrow stromal cells (BMSC), and evaluable introduction of alternative therapeutic approaches.

Aim. The aim of this study was to investigate the role of bone marrow stromal cells in the course of donor marrow cells engraftment and their signifi cance in post-transplant

complications.

Materials and methods. The study included clinical observation of the post-transplant course in ten patients with acute myeloid leukemia (AML) and 7 healthy donors. Bone

marrow nucleated cells were selectively harvested two weeks prior to BMT, followed by monolayer culture in alpha-MEM culture medium with 20 % fetal bovine serum. Upon growth

of fi broblast-like cell colonies (CFU-F), their hematopoiesis-supporting activity was determined in the agar-drop/liquid culture system, as well as their diff erentiating ability along

adipogenic and osteogenic pathways. We have also analyzed the relative expression of selectin and CXCR4 genes in these cells.

Results. When comparing functional characteristics of BMSC from healthy donors and AML patients, an increased hemostimulatory activity of the latter was noted, as refl ected by an

increase in large and small CFU-GM numbers (p y correlated with platelet recovery time (P = 0.05). In contrast, higher numbers of osteogenic colonies in culture were associated with

an increased time to leukocyte lineage recovery (P = 0.05). When analyzing gene expression in BMSC population, a decreased expression of the CXCR4 gene responsible for the homing

eff ect, with age of the patient’s (P = 0.05) was noted. The selectin gene expression in BMSC was higher by AML patients as compared to healthy donors.

Conclusion. Stromal cells derived from the patients’ bone marrow exhibit higher proliferative activity and marked expression of molecules mediating HSC homing, as compared

with a group of healthy donors. That fi nding could be explained by aff ection of stromal cells by previous chemotherapy and myelosuppression. BMSC from AML patients taken before

bone marrow transplantation are characterized by a more pronounced capacity to osteogenic and adipogenic diff erentiation than those from healthy donors. Increased adipogenic

diff erentiation ability of the BMSCs is associated with a more rapid recovery of hematopoiesis.

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A S T R A C T N O . : O - 2 9 6

Feasibility and outcome of allogeneic hematopoietic stem cell transplantation with post-

transplant cyclophosphamide in children with acute leukemia. Single centre experience

O.V. Paina, A.S. Borovkova, P.V. Kozhokar, S.V. Razumova, K.A. Ekushov, O.A. Slesarchuk, I.S. Moiseev,

O.V. Pirogova, A.L. Alyanskiy, E.V. Babenko, L.S. Zubarovskaya, B.V. Afanasyev

First Pavlov State Medical University of St. Petersburg, Russia

Key words: post-transplant cyclophosphamide in children, allo-HSCT

Introduction. Allogeneic hematopoietic stem cell transplantation from matched related, unrelated or haploidentical (allo-HSCT) donors (MRD, MUD, HaploD) are often the

only method of radical treatment of patients suff ering from hematological malignancies. The development of severe graft-versus-host (acute and chronic) disease (aGVHD;

cGVHD) determines the length of hospitalization and signifi cantly contributes to the structure of mortality, despite the use of new modes of conditioning regimens and modern

pharmacological drugs (calcineurin inhibitors (CNI): cyclosporin A – CSA, tacrolimus – Tacro; m-TOR inhibitors – Sirolimus - Sir). At the present time the John Hopkins and Fred

Hutchinson Cancer Research Center groups evaluate the effi cacy of high dose (50 mg/kg) cyclophosphamide administered on day 3 and 4 (PTCY) after allo-HSCT as a new option

and have obtained the current satisfactory results in adults, but its feasibility is not clear in children.

Aim. In the current study, we retrospectively evaluated the feasibility and outcome of PTCY based allo-HSCT (MRD-HSCT, MUD-HSCT and Haplo-HSCT) in children with acute leukemia

employing chemotherapy based conditioning regimen (MAC and RIC). Primary end points: engraftment rate, aGVHD, overall survival (OS). Secondary end points: relapse rate,

non-relapse mortality (NRM).

Materials and methods. 132 children (range from 0–19 y.o., median 8 y.o.) with AL (ALL – 78 pts, AML – 52 pts, JMML – 2 pts) were included. PTCY-group – 84 pts

1st or 2nd remission – 33 pts, resistant disease – 50 pts). Types of HSCT: MRD-HSCT – 1 pts, MUD-HSCT – 25 pts, Haplo-HSCT – 57 pts. Conventional prophylaxis of GVHD (control-

group) – 49 pts (1st or 2nd remission – 15 pts, resistant disease – 34 pts), types of HSCT: MRD-HSCT – 4 pts, MUD-HSCT – 6pts, Haplo-HSCT – 39 pts). Conditioning regimen: MAC +

ATG received 29 pts, MAC + PTCy 50 mg/kg on D+3, +4 in 36 pts. RIC + ATG received 20 pts, RIC + PTCy 50 mg/kg on D+3, +4 in 47 pts. All pts PTCy-group had prophylaxis of aGVHD

based on calcineurin inhibitors (CSA or Tacro) with or without MMF or Sir since D+5. The control group had prophylaxis of aGVHD by CNI as well with MMF and methotrexate – 49 pts.

The median follow-up of surviving patients was 13.5 months. Statistical analyses were performed using SPSS V19.

Results. 73.5 % of patients achieved engraftment in PTCY-group vs 81.6 % control-group (P = 0.4). Cumulative incidences of aGVHD grate II–IV (19.7 % vs 6 %, P = 0.000) was

signifi cantly lower in PTCY-group compared to control-group. Cumulative incidence of relapse (44.5 % vs 50 %, P = 0.7), NRM (14.8 % vs 25 %, P = n.s.) were not statistical signifi cant.

One-year overall survival was the same in both group (PTCy-group – 54.2 % vs 53.1% in control-group, P = 0.5).

Conclusion. Allo-HSCT with PTCY in acute leukemia is a safety and feasibility option. PTCY in combination with CNI and MMF or sirolimus had lower incidence of aGVHD. Future

studies are needed to increase OS and reduce relapse rate in post-transplant period.

A B S T R A C T N O . : P P - 3 2 7

High mixed chimerism in CD3 cell line in patients with AML after allogenic HSCT

V. Brilliantova, L. Shelikhova, Zh. Shekhovtsova, E. Gutovskaya, I. Shipitsina, D. Shasheleva, D. Balashov, A. Livshits,

R. Khismatullina, M. Persiantseva, M. Ilushina, E. Raikina, V. Bobrynina, M. Maschan, A. Maschan

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: chimerism, acute myeloid leukemia, CD3+ cells

Introduction. Hematopoethic stem cells transplantation (HSCT) is the only way to save a life in patients with many diseases, especially in onkohematology. The most common

complications after HSCT are infections, allograft rejection, graft-versus-host-disease (GVHD) or relapse of disease. To observe the process of transplant functions measuring of donor’s

chimerism (a number of donor’s cells) in peripheral blood and bone marrow is often used in patients received allogenic HSCT. After HSCT with TCRab depletion a long-term high mixed

chimerism may observed in some patients in CD3 cell line. It is important to understand, how high mixed chimerism in CD3 cell line aff ects on post transplant period.

Aim. Research study of donor’s chimerism in CD3 cell line in patients with AML received allogenic HSCT

Materials and methods. In this study was observed 27 patients with AML, received HSCT since May 2012 till October 2014. 11 patients received haploidentical transplantation and

16 patients receaved transplantation from HLA-matched unrelated donor. Measuring of donor’s chimerism proceeds each 30 days during 1 year after HSCT. During this time 11 patients

had a relapse. CD3 cells were isolated on dynabeads (applied biosystems) from bone marrow samples. Chimerism was detected by using Insertion/Deletion polymorphisms (RTQ PCR).

Results. The research study of donor’s chimerism in CD3 cell line has detect the group of patients with high mixed chimerism (recipient cells is more than 40 %). However, patients

in this group that didn’t have relapse all received HSCT from unrelated donor. In patients, received HSCT from haploidentical donor, number of recipient’s cells more than 15 % in CD3

cell line was observed only in patients with relapse.

Conclusion. High mixed chimerism (more than 40 %) in CD3 cell line in patients with AML after HSCT from unrelated donor is not associated with relapse, comparing with patients

received HSCT from haploidentical donor. Patients with haploidentical donor and more than 15 % of recipient’s cells in CD3 cell line all had relapse.

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A B S T R A C T N O . : P P - 3 4 5

Breastfeeding is not contraindicated in patients undergoing HSCT

D. Skobeev1, E. Skorobogatova1, K. Kirgizov1, 2

1Russian Children’s Clinical Hospital, Moscow, Russia;2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: Breastfeeding during, stem cell transplantation,post-transplant period.

Introduction. In spite of current conceptions and recommendations on children’s nutrition with HSCT, the question about breastfeeding remains open and controversial for now.

Aim. We aimed to show the possibility of breastfeeding during the HSCT for pediatric patients.

Materials and methods. 20 patients with HSCT at the BMT unit of the Russian children’s clinical hospital were allowed to be breastfed between 2012 and 2015. The average age

of the children was 18 months (between 7 and 30 months). Types of HSCT: Autologous 30 % (n = 6), Allogenic 70 % (n = 14): MUD 40 % (n = 8), MSD 15 % (n = 3), haploidentical

15 % (n = 3). All pts. after AutoHSCT were with neuroblastoma (30 % from all pts.) received conditioning regimen with Treo 42 gr/sq.m. + Melphalan 140 mg/sq.m. All patients with

Allogenic HSCT received full intensity Treo-based conditioning regimen. Diagnosis: AML – 30 % (n = 6), Hurler syndrome – 20 % (n = 4), SCID – 15 % (n = 3), CGD – 5 % (n = 1).

Breastfeeding was not terminated during the conditioning period and within the whole early post-transplant period with complications.

Results. The incidence of severe oropharyngeal mucositis and neutropenic enterocolitis (higher than grade 2) in all the survived patients (n = 20) was 15 %. There were no severe

infectious complications and inoculation of de novo pathological microfl ora that serve as indicators of breastfeeding complications observed in the analysed group. The incidence of

intestinal form of graft versus host disease (GvHD) with allogenic HSCT (more than II gr.) was 14.3 %.

Conclusion. The performed analysis demonstrated that breastfeeding may be recommended for children at the BMT unit as it doesn’t aff ect the process of early post-transplant

period and doesn’t increase the incidence of toxic and immune complications, protected gastrointestinal infectious complications and is the important element of psychological

support in the “mother-child” system in a clinical setting.

A B S T R A C T N O . : O P - 3 4 8

Reduced-intensity versus myeloablative conditioning allogeneic hematopoietic stem cell

transplantation for patients with Hurler syndrome

A. Borovkova1, K. Kirgizov2, E. Skorobogatova3, O. Paina1, P. Kozhokar1, S. Razumova1, Yu. Fedyukova1,

E. Pristanskova3, N. Sidorova3, V. Konstantinova3, A. Osipova1, K. Ekushov1, E. Semenova1,

L. Zubarovskaya1, A. Maschan2, B. Afanasyev1, A. Rumyantsev2 1Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical

University of St. Petersburg, Russia; 2Federal Research Center of Pediatric Hematology, Oncology and Immunology named afterDmitriy Rogachev, Moscow, Russia; 3Russian Children’s Clinical Hospital, Moscow, Russia

Key words: allogeneic hematopoietic stem cell transplantation, Hurler syndrome, children, RIC, MAC

Introduction. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is still the treatment of choice for children with severe form of mucopolysaccharidosis type I (Hurler

syndrome). The preparative regimen given to those patients is mostly myeloablative conditioning (MAC). But using of MAC is limited for patients with multisystem involvement and

low Lanskoy score. Reduced intencity conditioning regimens (RIC) are associated with lower rates of severe toxicity, from another side, RIC is the risk factor for graft failure in Hurler

syndrome (HS) patients.

Aim. To compare effi cacy of allo-HSCT with RIC versus MAC in children with Hurler syndrome.

Materials and methods. From 2004 to 2015 unrelated allo-HSCT were performed in 31 patients with HS. Median age at the time of diagnosis was 15 months (3–24 months),

at transplantation – 22 months (from 9 months to 42 months) (RIC – 28 months, MAC – 18 months). Five patients (16 %) received allo-HSCT from HLA-matched related,

26 (84 %) – from matched (n = 23) or partially matched (9/10) (n = 3) unrelated donors. The donor sources were bone marrow (BM) (n = 19, 61.3 %), peripheral blood stem cells (PBSC)

(n = 10, 32.2 %) or cord blood (n = 2, 6.5 %). The myeloablative (Busulfan or Treosulfan-based) and reduced-intensity (consisted of Fludarabine, Melphalan and ATG) conditioning

regimens were used in 23 (74 %) and 8 (26 %) patients respectively.

Cyclosporine A or Tacrolimus plus short course of methotrexate or MMF were used for GVHD prophylaxis. In 3 cases immunomagnetic СD3/СD19+-depletion of PBSC (by CliniMACS)

was used additionally.

Results. Median follow up was 50 months (range 7– 140 months). 27 patients are alive. Overall survival – 69.5 ± 0,16 % (MAC – 72.75 %, RIC – 75 %, P = 0.24). The median time

to neutrophil recovery > 0,5 × 109/L was 20 days (11–29). All patients were engrafted with achievement of full donor chimerism and normal alpha-L-iduronidase activity in leucocytes

on D+30. In 7 cases (2 (25 %) after RIC and 5 (22 %) after MAC, P = n.s.) decreasing of donor chimerism was observed. Graft rejection was observed in 5 (16 %) patients (MAC – 4 (17 %),

RIC – 1 (12.5 %), P = n.s.). During RIC alloHSCT none patients developed severe toxicity (grade III–IV according to NCI CTC ver. 3.0 criteria). Seven (30 %) patients after MAC had

mucositis grade III. Results of hepatic tests did not diff er signifi cantly between the two groups. Seventeen (74 %) patients in the MAC group had grades II–III skin toxicity compared

with none in the RIC group (P = 0.002). There were no statistically signifi cant diff erences in acute and chronic GVHD rates.

The main reason of death in the MAC group was lung infection. One patient in the RIC group died from acute GVHD, 1 patient died due to TRALI-syndrome.

Conclusion. Our data demonstrate that RIC regimens resulted in comparable overall survival rates with MAC regimens in Hurler syndrome patients. MAC was associated with higher

risk of severe mucositis and skin toxicity. There was no statistically signifi cant diff erence in rejection rate between patients conditioned with MAC and those conditioned with RIC.

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A B S T R A C T N O . : O P - 3 7 3

Single-center study of outcome of hematopoietic stem cell transplantations:

10/10 versus 9/10

N. Sidorova1, K. Kirgizov1, D. Balashov2, P. Trachtman2, M. Persiantceva2, E. Pristanskova1, V. Konstantinova1,

O. Blagonravova1, M. Maschan2, E. Skorobogatova1, A. Maschan2

1Russian Children’s Clinical Hospital, Moscow, Russia; 2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: HSCT, ALL, AML, MUD, MMUD, a.GVHD, Tacro/Mtx scheme

Introduction. MUD HSCT is routine method now. Importance of HSCT from 9/10 and 10/10 MUD is discussed.

Aim. To compare the effi cacy of allo-HSCT from MUD (10/10) and MMUD (9/10) for children with hematological malignancies.

Materials and methods. 97 HSCT from 9/10 and 10/10 MUD performed during the period 2003–2014. Diagnosis: AML – 68 % (n = 66), ALL – 32 % (n = 31). Gender distribution:

male – 71 % (n = 69), female – 29 % (n = 28). Age median – 8.3 y.o. (1–17 y.o.). Stem cell source: BM – 80 % (n = 77), PBSC – 20 % (n = 20). 77 pts. (79 %) received 10/10 MUD HSCT

and 20 pts. (21 %) – 9/10 MUD HSCT. Conditioning regimen contained diff erent agents due to protocol and type of disease. The type of a.GvHD prevention therapy carried cyclosporin

A (CsA)/methotrexate (Mtx) until 2007, tacrolimus (Taсro)/CellCept (MMF) from 2007 to 2011 and from 2012 used a combination Takro/Mtx.

Results. Overall incidence of a.GvHD (I–IV st.) in 10/10 group was 69 % (n = 53) and in 9/10 – 80 % (n = 16), a. GvHD (III–IV st.) incidence – 16 % (n = 12) and 30 % (n = 6)

accordingly. We revealed that type of a.GvHD prevention therapy can signifi cantly improve outcome: Tacro/MTX prevention better in comparison with other types (Tacro/MMF and

others). In both groups during the Tacro/MTX scheme have been no cases of a. GvHD (III–IV st.). Infection’s episodes weren’t signifi cantly diff erent in 9/10 and 10/10 groups. In 10/10

group at median follow up of 12.2 years, the estimated probability of OS was 56 % and in 9/10 group OS was 30 %, median follow-up – 11.8 years.

Conclusion. Our results suggest that 10/10 transplants have better outcome and lower incidence of severe a. GvHD. Tacro/MTX scheme can decrease a.GvHD (I–IV st.) episodes in both

groups. Our experience showed that 9/10 results improved in past several years. Now 9/10 transplants is good option in case of absence 10/10 donor.

A B S T R A C T N O . : O P - 3 7 5

The effi ciency of the conditioning regimen at hematopoietic stem cells transplantation

from HLA-matched (10/10) and mismatched (9/10) unrelated donors for children

with hematological malignancies

N. Sidorova1, K. Kirgizov1, D. Balashov2, P. Trachtman2, M. Persiantceva2, E. Pristanskova1, V. Konstantinova1,

O. Blagonravova1, M. Maschan2, E. Skorobogatova1, A. Maschan2 1Russian Children’s Clinical Hospital, Moscow, Russia; 2Federal Research Center of Pediatric Hematology,

Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: HSCT, ALL, AML, a. GVHD, MUD, MMUD, MAC-Treo

Introduction. Hematopoietic stem cell transplantation (HSCT) from unrelated donors is an accepted treatment of patients with oncohematological diseases. Probability of

identifi cation of HLA-matched donor is only 70–75 %.

Aim. To evaluate the effi ciency of the conditioning regimes used in HSCT from HLA-matched (10/10) and mismatched (9/10) unrelated donors for children with hematological

malignancies method of retrospective analysis.

Materials and methods. The study included 97 HSCT from unrelated donors performed in the period 2003–2014. 71 % (n = 69) were male, while 31 % (n = 28) were females.

The age of patients 1–17 years (median 8.3 years). Nosological structure: AML – 68 % (n = 66), ALL is a 32 % (n = 31). The source of stem cells: BM – 79 % (n = 77), PSCC – 21 %

(n = 20). 79 % (n = 77) patients, HSCT was performed from HLA-matched (10/10) unrelated donor and 21 % (n = 20) from mismatched (9/10). At the time of HSCT was 65 %

(n = 63) patients were in complete remission and in 35 % cases (n = 34) noncomplete remission. Conditioning regimes: MAC-TBI 2 % (n = 2), MAC-Treo 17 % (n = 16), MAC-Treo-

Mel 32 % (n = 31), MAC Bu 5 % (n = 5), MAC Bu-Mel 41 % (n = 40) and RIC 3 % (n = 3). GVHD prophylaxis was performed with cyclosporine A(CsA)/methotrexate(Mtx) until 2007,

tacrolimus (tacro)/CellCept (MMF) from 2007 to 2011, and since 2012 has used a combination of tacro/Mtx.

Results. The best score by level of overall survival was detected in patients receiving HSCT from a 10/10 donor, after conditioning the MAC-Treo was 85 % (n = 11). Overall survival

of patients after HSCT 10/10 and MAC-Bu-Mel 54 % (n = 15), MAC-Treo-Mel 50 % (n = 14), RIC 33 % (n = 1), MAC-Bu 25 % (n = 1) and MAC-TBI 0 %. When performing HSC

transplantation from mismatched unrelated donors 9/10 overall survival was: MAC-Treo 33 % (n = 1), MAC-Bu-mel 33 % (n = 4), MAC-Treo-mel 33 % (n = 1), RIC 33 % (n = 1),

MAC-Bu 0 %, MAC-TBI 0 %. The level of the two-years relapse free survival of patients after HSCT from 10/10 and 9/10 donor was 56 % (n = 43) and 30 % (n = 6), respectively.

Conclusion. By performing HSCT from HLA-matched (10/10) and mismatched (9/10) unrelated donors the choice of conditioning regimen infl uences at the HSCT outcome. The use of

MAC-Treo associated with better OS and two-years RFS, and is the best option in HSCT from an unrelated donors in children with hematological malignancies.

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Venous access in treating children with cancer: a 6-year experience of a single institution

M. Rykov, V. Polyakov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: pediatric oncology, chemotherapy, venous access, implantable venous ports, central vein catheterizations

Introduction. The treatment of any oncologic disease is impossible without a venous access. What kind of properties should it possess? It has to be safe, easy to use, implanted only

once during the treatment course and have minimal risks associated with implantation and use.

Aim. Prevention of complications of intravenous chemotherapeutic agent administration.

Materials and methods. From 2010 to 2015 we were monitoring the treatment of 2286 children (3 months – 17 years) with oncologic diseases. 2099 (91.8 %) patients underwent

3930 subclavian vein catheterization, 187 (8.2 %) patients – 187 venous ports implantations.

Results. When installing the subclavian catheters intraoperative ultrasound navigation was performed in 23 (0.6 %) cases, intraoperative fl uoroscopy was not used.

For the implantation of port systems, ultrasound and fl uoroscopy navigation used in all cases. Complications and technical diffi culties during catheter insertion were observed

in 98.3 % of cases, during venous port implantation – in 23 % of cases. Complications of subclavian catheter and venous port use were observed in 97.3 % and in only 11 % of cases,

respectively. Subclavian catheters compromised cancer treatment in 45.9 % of patients, implantable venous ports – in 1.7 % of patients. Each patient with a subclavian catheter

underwent central venous catheterization 4 to 19 times (mean 6 times) during treatment. Catheter dwell time exceeded the recommended limit in all patients except for cases of

catheter removal by patients. On multiple occasions all patients were discharged with a subclavian catheter in place.

Conclusion. Venous ports obviously match the criteria mentioned in the introduction. Subclavian catheter use resulted in cancer treatment protocol deviation in almost 50 % of cases,

thus leading to a poorer prognosis and signifi cantly increasing the number of invasive procedures and instances where general anesthesia was needed.

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Venous access in the treatment of children with cancer: results of a multicenter study

M. Rykov1, N. Grigorieva2, A. Ulanova2, I. Volihin2, V. Kochkin3, I. Turabov2, V. Polyakov1 1Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia;

2Arkhangelsk regional children’s clinical hospital named after P.G. Vyzhletsov, Russia; 3Russian Children’s Clinical Hospital, Moscow, Russia

Key words: pediatric oncology, venous access, implantable venous port-systems, subclavian catheters

Introduction. The results of Russia’s fi rst multicenter study of the use of venous port systems for the treatment of patients with cancer.

Aim. Prevention of complications of intravenous chemotherapeutic agent administration at pediatric oncology.

Materials and methods. From 2006 to 2015 we were monitoring the treatment of 463 children (aged 3 months to 17 years) with oncologic diseases. This patients underwent venous

port implantations (IVPs).

Results. In 463 cases during insertion of 463 IVPs the following complications and technical diffi culties were present.

1. Unintended puncture of the common carotid artery (CCA) during the puncture of the IJV – 19 cases (4.3 %).

2. Retrograde positioning of the distal end of the guidewire in the IJV – 67 cases (14.4 %).

3. Placement of the distal end of the guidewire into the punctured SV – 35 cases (7.6 %).

4. Diffi culties driving the guidewire into the IJV after successful puncture – 43 cases (9.3 %).

5. Retrograde port catheter positioning in the IJV during ECG-guided implantation – 8 cases (1.7 %).

Despite the use of intraoperative fl uoroscopy, there have been cases of the retrograde positioning of the distal end of the guidewire in the IJV due to the peculiarities of topographic

anatomy of children. However, visual inspection in all cases facilitated intraoperative adjustments of these complications.

The use of 463 IVPs was complicated by the following.

1. Venous port contamination – 11 cases (2.5 %).

2. Occlusion of the IVP by a thrombus – 23 cases (5 %).

3. Subcutaneous fat layer thinning above the port chamber – 7 cases (1.7 %).

Venous port contamination was caused by violation of rules of operation – Huber needle clinicians used more than a month without replacement. This resulted in infection of the

tissues around the port chamber. This has resulted in the removal of the IVP. Subsequently, these patients were implanted port systems on the opposite side.

Conclusion. IVPs are safe, implanted only once during the treatment course and have minimal risks associated with implantation and use. Unfortunately, in the Russian port systems

are used only in several hospitals.

A B S T R A C T N O . : O P - 0 8 7

Possibilities of endosurgery in diagnosis of tumor diseases of thoracic

and abdominal localization at children

D. Rybakova, А. Kazantsev, P. Kerimov, M. Rubanskiy, A. Khizhnikov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: endosurgery, children oncology

Introduction. Use of endosurgery in the diagnostic purposes is directed on specifi cation of nature of the changes revealed at noninvasive methods of research, receiving enough

tumor material for all types of morphological research and an assessment of prevalence of process. Diagnostic endosurgery is successfully approved in adult oncology and there are

data on use of this method in children oncology in foreign literature.

Aim. To defi ne possibilities of endosurgery in diagnosis of tumor of thoracic and abdominal localization at children.

Materials and methods. in our institute endosurgery operations at patients with various tumor pathology are regularly performed since 2007. From 2007 to 2012 with the

diagnostic purpose it is carried out the 160th operation at 153 children, from which 63 laparoscopic and 44 thoracoscopic biopsies. Except diagnostic biopsies it was executed

53 thoracoscopic resections of lungs at 51 children for the purpose of diagnostics of progressing of a disease, confi rmation of metastatic defeat or infl ammatory process that made

33 % of all diagnostic operations.

Results. The age of patients varied from 2 months to 19 years. The ratio on a fl oor was approximately equal: boys was – 76 (49.7 %), girls – 77 (50.3 %). And children about one year

there were 9.8 % (15 children). Duration the thoracoscopic and laparoscopic biopsies made from 20 minutes to 260 minutes, on average 59 minutes. Blood loss during diagnostic

operations averaged 78.3 ml. Intraoperative complications are in 5 cases, and postoperative in – 8. After the endosurgery operations at 59.3 % of patients special treatment was

carried out, 7.4 % had a repeated operation, but already in radical volume and in 33.3 % were written out on continuation treatment in a residence or in profi le establishment. Terms

of an initiation of treatment of special treatment varied from several hours to 20–30 days because of complications during operation. The average time of the beginning of special

treatment made 4 days.

Conclusion. Advantages of use of endosurgery in children’s oncology are: early terms of the beginning of special treatment, small injury, the minimum blood loss, low number of

postoperative complications, early activation of the patient and reduction of terms staying in a hospital, good cosmetic eff ect. Performance the endosurgery of operations at children

with malignant tumors it is possible aged from several weeks, thus the oncological principles of performance of surgery aren’t broken, and also the age of the child isn’t the limiting

factor for performance the endosurgery of operations.

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Surgical treatment of children with solid pseudo papillary tumors of the pancreas

D. Rybakova, А. Kazantsev, P. Kerimov, M. Rubanskiy, A. Khizhnikov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: solid pseudopapillary tumor, laparoscopy, endosurgery

Introduction. Solid pseudopapillary pancreatic tumor (SPT) are rare neoplasms of low malignant potential, account for less than 3 % of the tumors.

Aim. To determine the tactics and the possibility of surgical treatment of children with SPT pancreas.

Materials and methods. From 2007 to 2015 operated on 11 children diagnosed with pancreatic SPT. We analyzed the clinical and diagnostic data, the volume of transactions,

the results of treatment and during follow-up.

Results. All patients were girls aged 9 to 15 years (mean age 12 years). The disease in most cases was bessiptomnoe, but have 2 children experienced pain in the epigastric region,

and 1 child had nausea and vomiting. According to a survey in 5 patients tumor was located in the tail of the pancreas, in 3 – body 3 and – in the head. The maximum size of the tumor

was 8.7 sm in diameter and located in the head of the pancreas. In 5 children underwent laparoscopic distal pancreatectomy, in 2 – gastropancreatoduodenal resection , from 2 –

central pancreatic resection, and 1 case is made distal subtotal resection of the pancreas, resection hooklike process pancreatic resection with duodenal wall. Operation time was from

90 to 290 minut maximum blood loss left 200 ml. Complications occurred in 6 patients: 4 cases – postoperative pancreatitis with pancreatic fi stula formation, bleeding in 1 pancreatic

branches of the splenic artery in 1 case – pneumonia. The follow-up of patients 8 years and 1 month. All patients were alive without evidence of disease recurrence.

Conclusion. SPT the pancreas is a rare disease in children, which usually occurs in girls’ puberty. The main treatment is surgery, the use of endosurgical possible, but very strictly

necessary to defi ne the indications for this type of treatment and the risk of postoperative complications.

A B S T R A C T N O . : O - 1 1 9

The use of a single endoscope access at neoplasms of internal genital organs in girls

V.B. Makhonin1, R.R. Bayramgulov1, 2, V.U. Sataev2, S.Yu. Muslimova2, V.A. Paramonov1, R. Hasanov2

1Republican Children Clinical Hospital, Ufa, Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, Republic of Bashkortostan

Key words: SILS, endosurgery, YST, myoma

Introduction. Pediatric endoscopic surgery continues to evolve, improving the quality of life of the patient in the postoperative period. NOTES technology was presented in a variety

of studies by many surgeons, however, SILS-technology in our opinion is the most appropriate for children. The technique uses a single port introducing through the umbilical ring for

the camera and 2 reticulators. This provides a minimally invasive approach, less traumatic and results in excellent cosmetic eff ect.

Aim. To assess the the possibility of using NOTES in surgical gynecology in children.

Materials and methods. 17 endoscopic surgical procedures for tumors of the internal genital organs in girls using a set of SILS-TM Port (Covidien) and specialized tools was done in

the clinic of pediatric surgery of the Bashkir State Medical University. Age of patients ranged from 12 to 15 years. All the girls standard preoperative examination was done.

The structure of diseases: cysts and benign tumors of the ovary – 14, myoma – 2 and malignant ovarian tumor – 1 case.

Results. Surgical treatment was performed under endotracheal anesthesia. Technology of single laparoscopic approach allowed carrying out complete surgical procedure in all cases.

The following operations were performed: cystectomy – 10, ovariectomy – 2, adnexectomy – 2, enucleation of the uterine myoma – 2 and separation of adhesions, resection of the

omentum and biopsy of the peritoneum (a second-look-operation in patient with YST after 3 cycles of chemotherapy) – 1 patient. Conversions were not observed. In the postoperative

period, there was no pain (narcotic analgesics not required), the lack of intra- and postoperative complications, reducing the time of staying patients in hospital (no ICU admissions,

discharge at day 3–4 after the operation), high cosmetic results.

Conclusion. Thus, the use of a single laparoscopic approach has allowed to perform operations in an adequate extent in girls with tumors of internal genital organs, and has improved

the results of treatment.

A B S T R A C T N O . : O P - 1 3 1

Bronchial blockers in pediatric thoracic surgical oncology

L. Martynov, N. Matinyan, A. Saltanov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: pediatric oncology, bronchial blockers, one lung ventilation, thoracic surgery

Introduction. In pediatric oncology, the lungs are the most frequent target of metastasis of various malignant tumors, so diagnostic and therapeutic surgeries are required. Currently,

video-assisted thoracoscopic surgery (VATS) techniques are performed for thoracic surgeries and biopsies, including mediastinum tumors. To guarantee optimal conditions for the

surgery, collapse of operated lung is required, thus one lung ventilation (OLV) should be performed. Until recently, the separation of the lungs in younger children was performed using

a single lumen endotracheal tube placed in main bronchus, in older children - with double-lumen tubes (DLT). The use of DLT in children can be traumatic and is often associated with

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pain in the throat and aphonia postoperatively. A newer devices for the lung separation, such as bronchial blockers (BB) Cohen, EZ-Blocker and Arndt allow to achieve lung collapse

avoiding traumatisation of trachea and other complications. Our experience of use of BB in older children is presented.

Aim. To improve the effi ciency and safety of lung ventilation in thoracic surgical oncology in children.

Materials and methods. During September 2014 – January 2016, 32 surgeries in patients 10–17 years old were performed for lung resection and biopsies. OLV was achieved and

mantained using BB. BB was introduced through the lumen of video endobronchial tube VivaSight-SL and was placed into the bronchus under continuous video control. Installation of

Arndt BB was conducted under endoscopic control. In 14 cases (43 %) the right main bronchus was blocked, in 18 cases (57 %) – the left main bronchus was blocked. Lung ventilation

was maintained in pneumoprotective mode. Time of BB installation, lung collapse score after installation of thoracoscopic ports, hemodynamics during surgery, the frequency of

postoperative complications such as sore throat and aphonia were evaluated.

Results. The mean time of intubation and BB installation was 255 ± 88 seconds. In all cases it was possible to achieve satisfactory lung collapse, but in 10 cases aspiration of

air through the channel of BB had to be performed. Ensuring the collapse of the right lung presents some diffi culties due to the anatomical and physiological features as higher

embranchement of the right upper lobe bronchus. In this case the use of EZ-Blocker BB is preferable as it is designed to provide stability with respect to carina, making it less likely to

displace during surgery. In surgeries of the left lung the use of Cohen BB is more preferable.

Conclusion. The use of BB require expensive high-tech equipment for video or endoscopic control, and the well trained staff with skills of BB installation and maintaining OLV.

Nevertheless, the use of BB in the thoracic surgery in pediatric oncology is a promising technique to achieve the eff ective collapse of the lung on the side of the operation with minimal

traumatisation, fewer complications postoperatively and rapid rehabilitation of patients after anesthesia.

A B S T R A C T N O . : P - 1 3 2

Superselective ophtalmic arterial chemotherapy for retinoblastoma in children

I. Letyagin, N. Milaschenko, L. Martynov, N. Matinyan, A. Saltanov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: retinoblastoma, ophthalmic artery, intraarterial melphalan, trigemino-cardiac refl ex

Introduction. Superselective ophthalmic artery injection of chemotherapy with melphalan has signifi cantly reduced the need for enucleation in patients with retinoblastoma.

Aim. To describe our experience with superselective ophthalmic artery chemotherapy (SOAC) in retinoblastoma and to report the serious adverse cardio-respiratory reactions

we have observed.

Materials and methods. Between February 2011 and March 2015 in Pediatric Oncology and Hematology Institute 54 eyes in 45 patients were treated. 17 patients with unilateral

retinoblastoma and 28 patients with bilateral retinoblastoma were included in the study. 104 cases of catheterization procedures were performed using a standardized protocol for

general anesthesia. All patients before procedure received heparin sulfate IV (30 IU-kg-1) and during procedure the same dose of heparin sulfate was administered.

Results. There were no deaths or major complications. Adverse cardio-respiratory reactions developed during 15 procedures (14 %). All reactions occurred during second or subsequent

catheterization procedures and were characterized by hypoxia, reduced lung compliance (the patients end tidal C02 (EtC0

2) decreased from 35 mmHg to 19 mmHg (normal 33–40 mmHg)

followed by a subsequent deterioration in the oxygen saturation (Sp02) to 70 % (normal 97–100 %). Hypotension (BP 54/20 mmHg) and tachycardia (130–150 bpm) were

also registered. The microcatheter was withdrawn and ventilation with 100 % oxygen was initiated. Adverse events were successfully treated during 10–15 min in all patients

by vasopressor support with Phenylephrine (0,05–1 mcg/kg/min) and atropine sulfatis administration. One procedure was abandoned due to prolonged hemodynamic instability.

5 patients required prolonged vasopressor support in early postoperative period. In 3 cases children had an acute ishemic stroke, which was confi rmed by MRI (treated quickly and

eff ectively).

Conclusion. Adverse cardio-respiratory reactions are commonly observed in SOAC for retinoblastoma. We believe that the adverse clinical signs represent an autonomic refl ex

response, akin to the trigemino-cardiac or oculorespiratory refl exes, and all patients should be considered at-risk. Reactions occur only during second or subsequent procedures

and can be life-threatening. The routine use of intravenous atropine does not seem to have altered the incidence or severity of these reactions. Anesthesiolgists and interventional

neuroradiologists involved in SOAC must be vigilant to ensure adverse reactions, when they develop, are treat them quickly and eff ectively. The symptoms described may represent

an acute onset of pulmonary hypertension mediated by release of vasoactive hormones or secondary to a vagal response triggered by stimulation of the ophthalmic artery. However,

further investigations are needed to improve the understanding of the manifestations, management, and clinical signifi cance of the described oculopulmonary refl ex occured only

during second or subsequent SOAC procedures.

A B S T R A C T N O . : O P - 1 3 6

Microsurgical reconstruction of maxilla and mandible in patients with head

and neck tumors – pediatric experience

N.S. Grachev1, S.V. Tereshchuk2, N.V. Babaskina1, I.N. Vorozhcov1, P.D. Pryanikov1, M.P. Kalinina1

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Main Military Clinical Hospital named after N.N. Burdenko, Moscow, Russia

Key words: microsurgery, free fl ap, maxilla reconstruction, mandible reconstruction

Introduction. 5 surgical operation with simultaneous microsurgical reconstructions of maxilla or mandible were performed in patients with head and neck tumors aged between

6 and 17 years in department of pediatric surgery and oncology in Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev.TREA

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Aim. Considering good results of free fl ap reconstruction in children we hope to encourage extension of microsurgical treatment in pediatric practice.

Materials and methods. 5 surgical operations were performed in patients with benign (3) and malignant (2) tumors of upper (1) and lower (4) jaws with simultaneous

reconstruction with fi bula (2) or ileac crest (3) osteomuscular free fl aps. In all cases arterial and venous anastomosis were made, and osseous parts of free fl aps were fi xed to intact

segments of jaw bones with medical hardware.

Results. Vascular anastomosis passability and free fl ap tissue viability were examined and proved in postoperational period in all cases. Postsurgical complications were revealed

in 2 of 5 cases, in both cases microsurgical reconstruction was performed with iliac crest free fl ap. First case required wound revision on second postoperative day because of

hematoma occurrence and fi nally resulted in wound contamination and partial necrosis of free fl ap bone part; in second case wound revision and fractional necrectomy were performed

on 14 postoperative day because of partial osteomyelitis of free fl ap bone. In both cases we succeeded in avoiding total free fl ap removal and after stepwise wound revisions with local

tissue reconstructions organ functionality was reached. Aesthetic and functional results of treatment including dental prosthetics in postoperational period promoted full social adaptation

of patients. Post operation follow-up period in the time of writing ranges from 2 months to 1.5 years, in all cases no evidence of disease recurrence or continued tumor growth was revealed.

Conclusion. Simultaneous microsurgical reconstruction allows combination of total tumor eradication and functional preservation that enables full rapid social adaptation of patient

which is extremely important in children. Good blood supply and functionality of free fl aps were maintained in all 5 cases including the two cases that required additional postoperative

surgical sanitizing and reconstructive interventions. In child adequate free fl ap nutrition allows synchronous graft grow with bordering tissues so that multiple corrective surgical

operations may become unnecessary to acquire optimal functional and aesthetic results.

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Minimally invasive surgery for the splenic lymphoma with splenomegaly

M.A. Teplyakova, A.V. Drey, V.A. Romanenko, O.I. Kit

Rostov State Medical University, Rostov-on-Don, Russia

Key words: minimally invasive surgery, splenomegaly, lymphoma

Introduction. Minimally invasive surgery has become the golden standard of care in the management of surgical diseases of the spleen. Such approach helps to reduce intraoperative

and postoperative complications. Also, use of the method decreases intraoperative blood loss, shortens patient’s recovery and hospital stay and can lead to better cosmetic outcomes.

Aim. Our goal was to determine the indications for the use of intracorporeal shrinkage technique during laparoscopic splenectomy, to introduce the method into practice

and to improve outcomes in patients with the splenic lymphoma.

Materials and methods. This method has been applied in 10 patients, 6 of whom were women and 4 men, the age of the patients was 27–35 years.

Results. Splenic size and weight remain the most important criteria of patient selection for minimally invasive splenectomy and often these characteristics predict the success of

laparoscopic splenectomy. Most authors report that size of craniocaudal axis more than 20–25 sm. is the limit of minimally invasive approach using. Spleen weight more than 2000

g. is the acceptable upper limit. For a successful management of patients with bigger spleen, hybrid technologies are used such as HALS (hand assisted laparoscopic splenectomy).

Our method allows abandoning the use of hand assisted port, which need for HALS approach. We have achieved this result by using of liposactor. А сannula of liposactor is inserted

thru one of the trocar punctures. After spleen mobilization and cutoff , the cannula is inserted into directly spleen pulpa. This procedure helps us to remove excess volume of tissue.

Further we can use methods of extracting the spleen application analogous to those that are carried out under standard procedures. Operation time is 90–120 minutes. Intraoperative

blood loss, on average, is 50 ml. The conversion was carried out in one patient (1 %).

Conclusion. Our original technique eliminates the additional port setting, saves time and cost of operations. This approach can also shorten patient’s recovery and hospital stay and

can lead to better cosmetic outcomes.

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Transnasal endoscopic surgery in children with and without image guidanceN.S. Grachev, I.N. Vorozhcov, S.S. Ozerov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: endoscopic surgery, CT-navigation

Introduction. Transnasal endoscopic surgery of neoplasms in children performed with and without CT-navigation.

Aim. To compare the quality of transnasal endoscopic surgical treatment of neoplasms of the nasal cavity, the nasopharynx, the paranasal sinuses (PNS) and the skull base (SB)

in children performed with and without CT-navigation.

Materials and methods. 71 patients aged between 22 days and 18 years who had undergone surgery of neoplasms of various degrees of surgical diffi culty were included into the

study. All patients were classifi ed into 2 groups: the primary one consisting of 36 children who had undergone surgery with CT-navigation (17 children had had a surgery of the 1st

degree of diffi culty (I) constituting the 1st subgroup and 19 children – a surgery of the 2nd degree of diffi culty (II) – the 2nd subgroup) and the control one made up by 35 children who

had undergone surgery without CT-navigation (19 children had had a surgery of the 1st degree of diffi culty (I) constituting the 3rd subgroup and 16 children – a surgery of the 2nd degree

of diffi culty (II) – the 4th subgroup). The statistical data analysis using Student’s t-criterion (P > 0.05) and Fisher’s exact F-criterion (P > 0.05) did not show any diff erences in patients’

gender and age distribution in the analyzed subgroups.

Results. The analysis of surgery duration and intraoperative blood loss volume in the 1st and the 3rd subgroups did not show any signifi cant diff erences (P = 0.96 and

P = 0.44 respectively, Mann–Whitney U-criterion). In contrast, the duration of surgery diff ered in the 2nd and the 4th group signifi cantly, with the diff erence comprising more than

30 min (P = 0.046, Student’s t-criterion), and the volume of intraoperative blood loss in the 2nd subgroup was signifi cantly lower as compared with the 4th subgroup

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There was no signifi cant statistical diff erence in the need for anterior nasal packing and the duration of nasal packing between the 1st and the 3rd subgroup (P = 0.28, Fisher’s

F-criterion, and P = 0.34, Mann–Whitney U-criterion respectively). In contrast, the need for anterior nasal packing in patients of the 2nd subgroup decreased signifi cantly (P = 0.002,

Fisher’s F-criterion) in regard to that one in patients of the 4th group. As for the duration of nasal packing, in case of the patients of the 2nd subgroup it was 3 times shorter than in case

of the patients of the 4th subgroup (P = 0.001, Mann–Whitney U-criterion).

We did not fi nd any signifi cant statistical diff erence in the median quantity of bed-days between the 1st and the 3rd subgroup (P = 0.076, Student’s t-criterion). In contrast,

the diff erence between the 2nd and the 4th subgroup comprised 2 days on the average. The diff erence was statistically signifi cant (P = 0.02, Student’s t-criterion). The comparison of

morphological data showed that the frequency of a radical removal of benign neoplasms was signifi cantly higher in the primary group than in the control group (P = 0.02, Fisher’s F-criterion).

Conclusion. We think that the application of a CT navigation system in transnasal endoscopic surgery of neoplasms of the nasal cavity, the nasopharynx, PNS and SB in children is

justifi ed. The results of the study refl ect the effi cacy of this method in the context of complex surgical manipulations. The duration of a surgery and the volume of blood loss decrease

signifi cantly, the need for postoperative nasal packing is reduced leading consequently to a quicker rehabilitation of children.

A B S T R A C T N O . : O P - 2 9 9

Technology biopsy of tumors of the urinary bladder

Z. Sabirzyanova, A. Pavlov

Russian Scientifi c Center of Roentgenoradiology, Moscow, Russia

Key words: bladder, biopsy, rabdomyosarcoma

Introduction. Among the entities of the bladder in children usually fi nd primary malignant tumors, benign – are rare. Of malignant tumors sarcomas are more frequently

(fi bromyosarcoma and chondrosarcoma) and myxomas, and of benign fi broid are observed, fi broepetelioma, hemangiomas. Since most entities originate from the muscle wall

of the bladder “gold standard” for initial diagnostics and “second look” is open biopsy of the bladder.

Aim. We assessed the possibilities of diff erent minimally invasive bladder biopsy technologies depending on the localization of tumor in 16 patients with tumors of the bladder

in the age from 1.5 to 15 years.

Materials and methods. Of these 8 patients had got bladder rhabdomyosarcoma, 2 with lejomyofi broma, 2 with hemangioma, 2 with bladder cancer and 2 with papilloma.

On localization of 6 patients tumor was localized in the the bladder neck and posterior urethra, 5 in the triangle L'eto, 2 on the front wall of the bladder and in the area of the bottom,

3 on the side and back wall. According to the previously performed ultrasound and MRI tumors had its noninvasive growth in 6 cases, in the others it was in all bladder wall.

Results. Transurethral bladder biopsy proved to be informative in 7 cases with noninvasive tumors, as well as in the localization of rabdomyosarcomas in the posterior urethra.

In the bladder neck and the back wall tumors trans rectal biopsy under ultrasound control was informative in 4 patients from 5. 1 patient with tumor in the front wall was successfully

held percutaneous biopsy under ultrasound control. Thus, the traditional open biopsy of bladder tumors was necessary only in 4 patients.

Conclusion. Minimally invasive biopsy of tumors of the bladder may be performed. Transurethral biopsy should be used in cases of noninvasive tumors, as well as in the tumor

in the posterior urethra. Tumor of the bladder neck miniinvasive biopsy can be performed under ultrasound control transrectal, and front wall tumor should biopsied percutaneously.

A B S T R A C T N O . : P - 3 3 2

Specifi c features of ovarian surgery in girls with oncological and hematological diseases

R. Oganesyan1, S. Talypov1, E. Andreev1, N. Uskova1, N. Grachev1, D. Bizhanova2, S. Varfolomeeva1, D. Kachanov1 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Russian Children’s Clinical Hospital, Moscow, Russia

Key words: ovarian surgery, germ cell tumor, oncology

Introduction. According to diff erent statistics the incidence rate of ovarian lesions in girls vary from 1 to 5 %.

Aim. To estimate the possibilities of ovarian surgery in girls in oncohematological practice.

Materials and methods. Since 2012 we performed 30 operations on ovaries (approximately 4.4 % of all abdominal operations for the same period), 12 of which were in girls with

unilateral ovarian lesion which required radical operation or enucleation. From 30 operations 9 were removal of ovaries with tumors and ipsilateral tubes , 3 – enucleation of bening

ovarian tumors, 6 – biopsy of ovarian tissue for subsequent ovarian cortex cryopreservation prior to combined therapy in cases with high gonadotoxic risk, 6 – ovarian transposition

before radiation therapy. Others were performed as diagnostic measures or in acute surgical conditions.

When the tumor is highly suspicious for malignancy we adhere to the rule of strict execution of several important stages.

1. Tumoroophorectomy of aff ected side without destruction of capsula.

2. Biopsy of opposite ovary.

3. Omentectomy.

4. Revision of retroperitoneal space, biopsy of suspicious lymph nodes.

5. Sample of ascetic fl uid should be taken for cytological analysis.

Results. 30 % of all girls had radical operations on account of germ cell tumors.

In cases of histologically malignant germ cell tumors all girls were administred adjuvant chemotherapy in the context of MAKEI-96 protocol. In 4 cases there were bening tumors,

including bening cysts and mature teratomas.

There was 1 postoperative complication which had happened in 2 month after laparotomy, tumoroophorectomy in 10-month old girl with germ cell tumor of ovary. It was TREA

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strangulated intestinal obstruction, which required resection of small intestine. Specimens of ovarian tissue for ovarian cortex cryopreservation for future autotransplant were

harvested laparoscopically with neither postoperative complications nor delay of the oncological treatment.

Oophoropexy in girls with oncohematological diseases is quite specifi c procedure prior to radiation therapy. We performed such operation as independent operation or as a stage

of operation on the organs of small pelvis.

Conclusion. In regard to ovarian surgery it is very important to have multidisciplinary team of oncologist, pediatric surgeon, pediatric gynecologist, radiation oncologist and fertility specialist.

A B S T R A C T N O . : P - 4 0 2

Experience of treatment of newborns with tumours from 2003 to 2015

A.A. Podshivalin, G.E. Chigvintsev

Children’s Republican Clinical Hospital, Kazan, Republic of Tatarstan

Key words: experience, mass lesion, surgery, newborn

Introduction. Offi cial statistics shows the tendencies of growth of birth-rate, at that the growth of primary diagnosed cases is registered too. Leading positions in the structure

of disease taking the diseases of lungs and congenital malformations (CM). Increasing of CM is connected not only with true growth of pathology, but with improving of prenatal

diagnosing. The number of patients with multiple CM increased. This tendency is registered not only in Tatarstan Republic but in hole World. The number of tumours is growing

progressively. Diseases rate in Russia is 9.5–13 per 100 000 of children.

Average rate of every year registering children with tumours in Russia increased during the last decade on 20 % and reached 6.45 thousands.

Aim. Analysis of treatment of newborns with oncological diseases (tumours of diff erent etiology), treated in the department of surgery of newborns from 2003 to 2014.

Materials and methods. Ninety four patients with mass lesions of diff erent localization were treated and analyzed: lymphangioms – 12 (12.7 %) of patients, cystic ovary –

22 (23.5 %), lung sequestering – 11 (11.8 %), hepatoblastoma – 2 (2.1 %), cystic forms of duplications of tracheobronchial tree – 5 (5.3 %), drenal hematoma – 9 (9.5 %),

neuroblastoma – 17 (18.2 %), Wilm’s tumour – 2 (2.1 %), soft tissue lesion – 4 (4.2 %).

Results. All mass lesions were underwent surgery, in 98 % of cases the lesions were removed totally, in 2 % – biopsy was done. Patients were treated with surgeon and hematologist/

oncologist in the following. In 2 % of patients which underwent surgery the metastases were revealed.

Conclusion. Radical tumourectomy is the surgery type of choice at newborns. Treatment must be multidisciplinary including surgeons, neonatologists and hematologist/oncologist.

Following monitoring and control observations (ultrasonic and CT-scans) are obligatory in post-surgery period.

A B S T R A C T N O . : O P - 4 0 4

Summing up the results of endoprosthesis for children with bone sarcomas:

The East-European sarcoma group (EESG)

D. Nisichenko, A. Dzampaev, M. Kubirov, D. Hestanov, M. Aliev

N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: endoprosthesis, sarcoma, limb salvage

Introduction. Limb salvage using endoprosthesis is currently the gold standard for the treatment of bone tumors in children and adults. Modern implants allow to increase its length

with the growth of the child.

Aim. Our aim was to retrospectively analyze outcomes of tumor endoprosthesis for upper and lower limbs in primary and secondary implantations.

Materials and methods. 268 children were treated in our clinic from 2000 to 2012. We performed 291 operations, among them 265 primary and 26 revisionary. Three patients came

straight for reimplantation after treating in another clinic.

Research group includes boys: 145/268 (54 %), girls: 123/268 (46 %). The most frequent diagnosis were osteosarcoma – 74 % and Ewing saroma – 19 %.

Average age – 12.6 years, minimal age – 3.5 years, max. – 18 years. After 18 patients continues their treatment in adult department of our clinic.

Speaking about the quantity of endoprosthesis – we shared them for 2 huge groups: standard zones (n = 254) of aff ection and accordingly, typical kinds of endoprosthesis and second

group that includes rare kinds of endoprosthesis, such as diaphysis of humer, radialis, femur, tibia and two cases of distal tibia. As far as those operations took a rare place, we cannot

achieve reliable evidence. Further we excluded those facts from the discussion.

We used endoprosthesis made by Wright (USA), W. Link (Germany), MUTARS – Implantcast (Germany), ProSpon (Chech Republic), Stanmore (England).

Results. Average follow up – 39 months. Min – 2 days (as a result of thrombosis we had to make an amputation), maximum monitoring time – 159 months (more then 13 years).

Femur involvement was 144/268 (53 %): distal femur 114/144 (79 %), diaphysis – 2/144 (1.4 %), proximal femur 11/144 (7.8 %), and total femur – 17/144 (11.8 %).

Tibia involvement was 80/268 (30 %): proximal part – 92.5 %, diaphysis – 5 %, distal tibia 2/80 (2.5 %). Total humer was in 14/41 (34 %) cases.

Two-year overall survival – 65 %. Infectious complication we observed in 44/251 (15 %) cases: distal femur – 5.6 %, proximal tibia – 20.5 %, total femur – 17.3 %. Aseptic loosing

we observed in 52/291 (18 %) cases. Local relapse – 22/268 (8 %). Twelve patients (6.7 %) fi nished treatment or went on reendoprosthesis in adult department of our clinic –

by reasons of instability, infection fail or shorting of lower limb.

Conclusion. For making limb salvage operations for children with bone sarcoma and do not yet reached skeletal immature we need to plan operations carefully and consider age of

patient and possible further reoperations and disease prognosis.

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A B S T R A C T N O . : O P - 1 3 9

The fi rst Russian experience of TomoTherapy application

for total body irradiation in children

D.A. Kobyzeva

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: tomotherapy, total body irradiation, bone marrow transplantation

Introduction. Total Body Irradiation (TBI) combined with chemotherapy is widely used all over the world as a conditioning regimen before hematopoietic stem cell transplantation

in patients with hematological malignancies. The main eff ects of TBI are elimination of tumour cells, as well as immunosuppression. The combination of TBI with chemotherapy

showed the best results of survival of patients compared to the conditioning regimen including only chemotherapy (Rinden et al., 1996; Kroger et al., 2001). The main issue of this

type of radiation therapy is a maximal homogeneous irradiation of the whole human body in an adequate dose. However, the irradiation of healthy organs and tissues that are highly

sensitive to ionizing radiation (such as lungs, kidneys, anterior eye) may lead to the development of serious and most often fatal complications (Cheng et al. 2008; Esiashvili et al.,

2009; Gerstein et al., 2009; Kal et al., 2009).

Aim. The development of TBI technique in children as a conditioning stage before allogenic bone marrow transplantation on TomoTherapy unit in order to maximal homogeneous

radiation of the target (Planning Target Volume, PTV) and reducing the dose on critical organs without worsening of the therapy results.

Materials and methods. Over the period from July, 2014 to February, 2016 radiation therapy was performed for 39 patients. Gender ratio: boys – 25 (64 %), girls – 14 (36 %).

The age of patients was from 3.2 to 20.3 years. Age median – 6.5 years. In the group there were patients with malignant hematological diseases: acute lymphoblastic leukemia

(n = 30), acute myeloid leukemia (n = 7), juvenile myelomonocytic leukemia (n = 1), myeloid sarcoma (n = 1). All patients were included in the group of high risk on the main disease.

The therapy was performed every day, 2 times a day in a single boost dose (SBD) on PTV 2.0 Gy to total boost dose (TBD) 12.0 Gy. Considering a potential sensitivity of healthy

organs and having based on a literature data the following organs of risk were chosen: lungs, kidneys and lens. The prescribed dose on PTV – 12.0 Gy. Prescribed dose on lungs:

Dmin

(minimal dose) – 6.0 Gy.

Results. After design of treatment plan on the TomoTherapy system the following result were received: the mean dose for PTV (Dmean

) was from 11.95 to 12.7 Gy,

(12.39 Gy is mean dose for all patients). The dose on critical organs were following: lungs – Dmin

from 5.7 to 7.1 Gy; V8 was from 5.5 to 40 % (mean for all patients – 25 %); kidneys – from

8.39 to 10.71 Gy (mean for all patients – 9.17 Gy); lens – from 1.43 to 7.71 Gy (mean for all patients – 5.13 Gy). ALL patients ended the planning therapy program. Radiation therapy

was performed in the course of the scheduled appointment of antiemetic therapy (5-HT3-receptor antagonists). Acute toxicity was observed in 38 (97 %) patients in the form

of nausea, vomiting (in 17 (55 %) patients – I degree, in 20 (51 %) – II degree, in 2 (5 %) – III degree on RTOG scale). Also during the treatment the acute parotiditis was observed

in 17 (44 %) of 39 patients.

Conclusion. The performance of TBI on TomoTherapy unit gives an opportunity to provide a maximal control over irradiation dose distribution in PTV with a simultaneous reduction

of dose on critical organs. It allows to decrease the probability of development of complications and to achieve high effi ciency of the treatment.

RADIATION ONCOLOGY

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A B S T R A C T N O . : O - 3 1 0

Helical tomotherapy for Askin’s tumor of chest wall: clinical outcomes

Siddhartha Laskar, Nikhil Kalyani, Nehal Khanna, Tushar Vora, Sajid Qureshi, Girish Chinnaswamy,

Seema Medhi, Mukta Ramadwar, Sneha Shah, Rituraj Upreti, Purna Kurkure

Tata Memorial Hospital, Mumbai, India

Key words: Askin’s tumor, chest wall, helical tomotherapy, outcomes

Introduction. Askin’s tumor of the chest wall is a challenging tumor to treat in view of the large volume of disease at presentation & the proximity to critical structures like the lungs,

heart, & spinal cord.

Aim. To evaluate the clinical outcomes of patients with Askin’s tumor of the chest wall treated using helical tomotherapy.

Materials and methods. The treatment comprised multiagent chemotherapy (CTh) and local therapy in the form of surgery (Sx) & radiation therapy (RT) or defi nitive RT alone.

Results. Sixty-four pts. between 7–21 yrs (Median: 17Yrs) treated with radical intent between January 2008 – December 2013 were included. Most (63 %) were males. Fifty-three

(83 %) pts. had non-metastatic disease. Ipsilateral pleural eff usion (IPE) was present in 21 (33 %). Median tumor volume was 840cc. Forty-one (64 %) underwent Sx + RT while

23 (36 %) received defi nitive RT. The Sx margins were close/positive in 24 (59 %) with residual viable tumor in 32 (78 %) of the resected specimen. Median percentage necrosis

in the resected specimen was 85 % (Range 45–99). After a median follow-up of 24 mths the actuarial local control (LC), disease free survival (DFS), & overall survival (OS) were

74 %, 54 %, & 81 % respectively. Pts. with IPE had poorer LC (50 % vs. 84 %, P = 0.07) & DFS (42 % vs. 71 %, P = 0.02). Tumors with median volume more than 850 cc had inferior

LC (67 % vs. 80 %, P = 0.74) & DFS (58 % vs. 61 %, P = 0.89) compared to smaller volumes. Post-op RT resulted in superior LC (90 % vs. 32 %, P = 0.001), DFS (69 % vs. 33 %,

P = 0.003), & OS (84 % vs. 75 %, P = 0.35) compared to defi nitive RT. RT related late toxicities included Grade 2 pulmonary fi brosis in 1 (1.5 %) & Grade 2/3 pneumonitis in 4 (6 %).

In pts. undergoing Sx, percentage necrosis in the specimen of more than 85 % resulted in superior DFS (73 % vs. 66 %, P = 0.43).

Conclusion. Primary tumor volume, surgical resection, surgical margins, percentage necrosis & presence of pleural eff usion infl uenced disease outcomes. RT related toxicities

with the use of Helical Tomotherapy were not signifi cant. The combination of CTh, Sx, & RT resulted in superior outcomes for non-metastatic Askin’s Tumor.

A B S T R A C T N O . : O P - 3 6 7

Intensity-modulated radiation therapy in multimodality therapy

for head-and-neck soft-tissue sarcomas in children

A. Usychkina, D. Kobyzeva, D. Kachanov, A. Loginova, N. Loginova,

K. Fateev, M. Teleshova, S. Varfolomeeva, A. Nechesnyuk

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: IMRT, soft-tissue sarcomas, head and neck

Introduction. Head and neck soft-tissue sarcomas in children are difficult to treat with radiation therapy due to their proximity to the critical organs and normal tissues.

Aim. To report the preliminary results of Intensity-Modulated Radiation Therapy (IMRT) for head-and-neck soft-tissue sarcomas (STS) in children.

Materials and methods. From 02.2012 to 12.2015 sixty-seven patiens with STS received external-beam radiation therapy as a part of multimodality therapy programme.

IMRT treatment was performed in 20 patients (29.8 %) with head-and-neck STS. The choice of treatment technique was based on the proximity of target volume to the critical

organs and normal tissues. The median patient age was 46.8 months (range, 6.7–155 months). Six patients (30 %) were younger than 3 years. Male to female ratio was 1.8 to 1.

The IRS clinical grouping was as follows: IRS II 2 patients (10 %), IRS III 13 patients (65 %), IRS IV 5 patients (25 %). STS histologic types were as follows: rhabdomyosarcoma

in 14 patients (70 %), non-rhabdomyosarcoma in 5 patients (25 %), rhabdomyosarcoma-like in 1 patient (5 %). The tumors were located in parameningeal, head and neck sites

in 12 (60 %), 7 (35 %) and 1 (5 %) patients, respectively. The surgical treatment consisting of R1, R2 resection and biopsy only was initial treatment in 2 (10 %), 2 (10 %) and 16 (80 %)

patients, respectively. All patients with histologic confi rmation of diagnosis received treatment according to the CWS-2009 guidelines. The time from the beginning of chemotherapy

to the beginning of radiotherapy was on average 8.73 weeks (range, 1.5–37.14). The planned total dose was from 41.4 to 54 Gy with normal fractionation of 1.8 Gy 5 times per week

according to the CWS-2009 guidelines. Two (10 %), 12 (60 %) and 3 (15 %) patients were treated to the total dose of 41.4 Gy, 50.4 Gy and 54 Gy, respectively. In two patients the

fraction dose was reduced to 1.6 Gy due to young age (1 year and 2 years). The total dose was 51.2 Gy and 49.6 Gy in these 2 cases.

Results. Nineteen patients (95 %) received the full planned total dose. One patient was treated to the reduced total dose of 46.8 Gy due to the high-grade acute hematological and

non-hematological toxicity (Grade 4) associated with acute systemic infection. In eight patients (40 %) treated with concomitant chemoradiotherapy the preventive tracheostomy

tube and/or gastrostomy tube was inserted based on the preliminary analysis of the acute toxicty risks. All patients had acute hematological toxicity Grade 1–4, acute skin toxicity

Grade 1 and 2 inside the irradiated area. Nineteen patients (95 %) had acute mucositis Grade 1–3. Five patients had acute esophagitis Grade 1–2. At the time of last follow-up

15 patients are alive (75 %), 4 patients (20 %) are lost to follow-up, 1 patient (5 %) died. One patient had the local tumor relapse in the regional lymphatic nodes outside of radiation

fi eld at 4.5 months follow-up. Another patient had treatment-resistant disease and clinical disease progression during the course of chemoradiotherapy. The patient died 5 months

after the start of treatment. In all other patients the irradiated tumors are controlled locally. The median follow-up time was 12.2 months (range, 1.27–35.7 months).

Conclusion. IMRT technique creates the homogenous dose distribution in the target volume and leads to the signifi cant dose-sparing of critical organs and normal tissues.

IMRT allows achieving the high local tumor control and low rate of Grade 3–4 acute treatment-associated toxicity in children with head-and-neck STS.

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A B S T R A C T N O . : P - 1 5 1

Prophylaxis of postanesthetic agitation syndrome

in young children with oncological pathology

S.V. Tumanyan, E.Yu. Semiletkina, A.A. Korobov

Rostov Scientifi c Research Institute of Oncology, Rostov-on-Don, Russia

Key words: agitation syndrome, sevofl urane, propofol, children, oncological pathology

Introduction. Therapy of oncological diseases involves a number of painful manipulations under anesthesia. Preschool and junior school children usually receive inhalation

monoanesthesia with sevofl urane with preserved spontaneous breathing without preliminary premedication. One of the side-eff ects of sevofl urane is so called agitation

or postanesthetic agitation syndrome (PAAS) developing in 6–80 % of cases. Among the causes of postanesthetic agitation syndrome (PAAS) development there can be mentioned

fast recovery of consciousness, insuffi cient analgesia, child’s agitated behaviour before anesthesia, child’s age, character of the performed operation, absence of premedication with

benzodiazepines, presence of central nervous system (CNS) pathology. For the purpose of arresting agitation and its prophylaxis it is recommended to use opioid analgetics, ketamine,

nitrous oxide, clonidine, benzodiazepines, propofol, dexmedetomidine. For a variety of reasons many of these medications can not be used in pediatric oncology when performing

short mask anaesthesia with sevofl urane.

Aim. To choose an optimal method of prophylaxis of agitation syndrome during inhalation anesthesia with sevofl urane with preserved spontaneous breathing in young children with

oncological pathology when performing short painful manipulations.

Materials and methods. The authors developed a questionnaire in order to detect children with perinatal CNS aff ection in anamnesis. 60 children aged 1–4 years with a minimal

cerebral dysfunction were included in the investigation. Patients were divided into 2 groups. In the 1st group (n = 30) painful manipulations were performed under mask inhatation

monoanesthesia with sevofl urane with preserved spontaneous breathing. Prevention of postanesthetic agitation syndrome (PAAS) was not performed in this group. In the second

group (n = 30) after conducting similar anesthesia in the setting of 100 % oxygen insuffl ation 1 % propofol (1–1.5 mg/kg) mixed with 0.9 % sodium chloride solution in a ratio of 1:5

was injected intravenously. In order to assess the effi ciency of the proposed method we used the defi nition of indices of systolic, diastolic, average dynamic arterial pressure, heart rate,

vegetation index Kerdo, SpO2, defi nition of the index of bispectral analysis of the electroencephalogram. The assessment of a sedation level was performed using the Ramsay scale.

Results. According to the results of the performed investigations the consequences of perinatal CNS aff ection were found in 65 % of children. Propofol injection prevented the

development of the agitation syndrome in 83.4 % of cases. Propofol normalized increased intracranial pressure, extended the phase of medicamentous sedation by approximately

12–16 min that gave the opportunity to restore the initial level of intracranial pressure when patients are asleep and decrease the intensity of painful impulsation.

Propofol injection caused the heart rate decrease on the average by 10 % that contributed to hypersympathicotonia suppression and normalization of vegetative tonus. 100 % oxygen

inhalation over 7–10 min after the end of anaesthesia improved the oxygenation of tissues, contributed to the prevention of hypoxia that develops due to the minor respiratory

depression. In 16.6 % of cases we did not obtain the expected result of propofol injection that was associated with the expressed painfullness of the performed manipulations. In these

patients the agitation syndrome was arrested with the repeated injection of propophol in combination with 50 % analgin.

Conclusion. Children with perinatal CNS aff ection in anamnesis belong to the risk group of postanesthetic agitation syndrome development. 1 % Propofol intravenous injection is

safe and prevents the development of agitation in 83.4 % of cases.

NEW DRUGS/EXPERIMENTAL THERAPEUTICS

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A B S T R A C T N O . : O P - 1 0 1

Atypical presentation, diagnostic challenges and outcome of dengue infection

in immnocompromised children with cancer: experience from India

Vasudeva Bhat, Shripad Banavali, Girish Chinnaswamy, Tushar Vora, Gaurav Narula, Maya Prasad, Sanjay Biswas,

Rohini Kelkar, Brijesh Arora

Tata Memorial Hospital, Mumbai, India

Key words: dengue, pediatric cancer

Introduction. Dengue Infection has varied immune-mediated clinical manifestations from minor self limiting fl u like illness to severe shock. There is very little knowledge about its

clinical presentation and outcome in immunocompromised patients with cancer and that prompted us to conduct this study.

Aim. To study the clinical profi le, diagnostics and complications in children with cancer infected with Dengue Virus.

Materials and methods. 78 patients with Dengue fever in children ≤ 15 years with malignancies on active chemotherapy at Tata memorial Hospital from September 2013

to September 2015 were retrospectively analysed.

Results. Sixty one (78 %) were males and mean age was 8.53 years (range, 1–15 years). Fifty fi ve (71 %) patients had a hematolymphoid malignancy with majority (42)

on intensive chemotherapy phase (Induction or consolidation). Fever was the most common and universal presenting complaint. Myalgia was second commonest symptom seen in

65 % of patients, fl ushing was seen only in 16 (21 %)patients and bone pains were uncommon. In all the patients, only NS1 antigen was positive and both IgG and IgM were negative.

Haemoglobin and hematocrit were in normal range in majority (97 %) of patients. Platelets on presentation ranged between 7–384 ×109/L which steadily decreased to a range of

4–232 ×109/L during the course of illness. Platelets recovered at a mean period of 7 days (range, 1–20 days). Platelet transfusions were required in 36 (46 %) patients; of which

25 (69 %) were refractory to platelets. Transaminits was common with mean AST and ALT values of 337 and 156 U/L respectively. CRP was low in 62 (79 %) with mean CRP value

1.78 mg/L (range, 0.02–19.2). Most common complication was ascites, which was seen in 23 patients (29 %) followed by pleural eff usion which was seen in 12 patients (15 %).

The most serious complication was Hemophagolymphohistiocytosis (HLH) seen in 10 (13 %) patients. There were fi ve deaths secondary to Dengue; two died of HLH and rest three

deaths due to immune reconstitution and capillary leak syndrome.

Conclusion. In immunocompromised children with cancer presenting with fever, a high suspicion has to be kept for Dengue fever during Dengue endemic seasons as fever may be the

only manifestation without classical fl ushing and bone or body pain. NS1 antigen should be used for diagnosis as antibody response is muted. Unlike general population, hematocrit

is normal is most children and platelet refractoriness is common. HLH is a common life-threatening complication and a high index of suspicion for HLH should be kept as timely use of

steroids may be life-saving. Late immune reconstitution may be fatal; hence, children should be admitted for a period after recovery of blood counts.

SUPPORTIVE CARE/PALLIATIVE CARE

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A B S T R A C T N O . : O - 1 6 0

Organization of nutritional support in a children’s cancer hospital

(the experience of our centre)

A.Yu. Vashura, E.S. Vasilyeva, Yu.V. Isarevich, D.V. Litvinov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: children, pediatric oncology, nutrition, algorithm of nutrition support

Introduction. Nutritional defi ciency and problems associated with it are registered with the incidence rate of 10–50 % in fi rst-time hospitalized children with oncological diseases.

During radiochemotherapy nutritional defi ciency is diagnosed in 75–80 % of patients, and after hematopoietic stem cell transplantation (HSCT) it is registered in the ovewhelming

majority of cases. The causes of nutritional defi ciency include tumour intoxication, radiochemotherapy toxicity, diseases of the digestive system, eating disorder. Subjective causes,

such as the delayed start of nutrition support (NS), inadequate adjustment of clinical nutrition, lack of motivation of treating physicians, exacerbate negative eff ects of these problems

and nutritional disorders themselves. Nutritional defi ciency increases the rate and intensity of complications, decreases survival rates, and makes treatment more expensive.

Aim. In practice, it is important to detect nutritional disorders timely, at early stages of special treatment, and to correct them adequately. Nutritional support is a mandatory

component of accompanying therapy at all stages of treatment and rehabilitation.

Materials and methods. The key principles of the organization of clinical nutrition in a children’s cancer hospital are creation of nutritional support groups (NSG) and an algorithm

of nutrition support (ANS). The aim of ANS is to optimize work associated with nutritional support of children at the children’s cancer hospital, increase its effi ciency, create and

improve the necessary compliance between a treating physician and a specialist of NSG. We developed an ANS that is a main fl owchart representing a certain sequence of actions and

detailed comments. These comments allows a treating physician to orient in a situation as fast as possible.

Results. The actions of ANS include: daily screening of nutritional status (clinical and laboratory, fi ndings, anthropometry); detailed nutritional assessment (bioelectrical impedance

analysis, indirect calorymetry) for indications; calculation of actual food consumption; daily nutrient and energy requirements; NS (enteral nutrition, parenteral nutrition, mixed

nutrition); monitoring of nutritional status (at least once a week); timely adjustment of recommendations that have already been made. Our long-term task appears to be a design

of software, that will include ANS.

Conclusion. An important part of the nutrition support is the interest and proper motivation of treating physicians, because otherwise the provision of adequate nutritional support

is impossible. Adherence to the formulated organizational and methodological principles is an essential prerequisite for provision of NS in pediatric oncology.

A B S T R A C T N O . : P P - 1 6 1

The results of screening for nutritional status of patients at a childrens’ cancer hospital

E.S. Vasilyeva, A.Yu. Vashura, S.S. Lukina, D.V. Litvinov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: pediatric oncology, malignancies, children, nutritional status, anthropometry

Introduction. Children who undergo treatment for oncological diseases have high risk of nutrition disorders. A series of studies has shown that nutrition defi ciency in oncological

patients reduces tumour cell responsiveness to radiation and chemotherapy, signifi cantly aff ecting treatment effi cacy and causing more severe side eff ects of antitumour therapy.

In view of this, the issue of state and dynamics of nutritional status of this category of patients seems to be of topical interest.

Aim. To analyze the screening results of the nutritional status of patients at hospitalization and during the course of chemotherapy.

Materials and methods. In the FRC PHOI n.a. D. Rogachev the screening of our patients’ nutritional status is carried out. The following anthropometric measures are estimated:

height, body mass, body mass index (BMI), upper arm circumference, triceps skinfold, upper arm muscle circumference (UAMC). The examination is carried out once a week, or more

often, if indicated. The assessment of the nutritional status also includes the investigation of the tissue composition of the body by the bioelectrical impedance analysis of patients

over 5 years old. The investigation results are entered into the electronic database and a case history (nutritional status assessment sheet).

Results. The analysis of nutritional status screening data has revealed that the majority of children at hospitalization in children’s cancer hospital have nutritional defi ciency. So, body

mass index (BMI) less than the 15th percentile at fi rst examination was registered in 38 % of children with retroperitoneal neuroblastoma, in 29 % of children with nephroblastoma,

in 37 % of children with CNS tumours, in 21 % and 35 % of children with acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) accordingly. Among the patients

with bone tumours (74 % of which were diagnosed with osteosarcoma, 21 % of children had Ewing sarcoma and 5 % – other types of bone tumours) BMI less than the 15th percentile

at hospitalization was observed in 32 % of patients. The largest amount of patients with nutritional defi ciency at the initial phase of study was registered among the children with

soft tissue sarcoma (54 %). We have also noted the lowering of upper arm muscle circumference indicator less than the 10th percentile in patients with nutritional status disorder,

that indicates a marked defi ciency of somatic protein pool. The analysis of nutritional status screening data over time on week 5 and 10 has shown that the majority of the patients

have negative dynamics of anthropometric measures caused both by the presence of main disease and by the infl uence of specifi c radiochemotherapy that was carried out. So, by

the 10th week of the study, the number of patients with BMI less than the 15th percentile in the group of children with neuroblastoma has increased from 38 to 49 %, in patients with

AML – from 35 to 43 %, in patients with nephroblastoma – from 29 to 43 %. In the group of patients with soft tissue sarcoma a number of children with nutritional defi ciency by

the 10th week remained considerable and amounted to 44 %. Upper arm muscle circumference indicators in these patients have also demonstrated negative dynamics that indicates

progressive defi ciency of somatic protein pool and muscle mass in patients in the setting of antitumour therapy being carried out.

Conclusion. The data we have collected demonstrate the need of correction of nutritional status in this category of patients as well as the advisability of preventive nutrition support.

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A B S T R A C T N O . : P - 1 7 9

Thrombus depositions as a risk factor for the development

of catheter-associated deep venous thrombosis in children

P.A. Zharkov, D.V. Fedorova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia

Key words: thrombosis, DVT, central venous line, CVL-related DVT, fi brin coat

Introduction. The presence of central venous line (CVL) is a risk factor for the development of deep venous thrombosis (DVT) especially in patients with malignant neoplasms (MN).

Except “true”, mural thrombosis, the presence of CVL in the central vein is often accompanied by the occurrence of the so-called “thrombus depositions” (TD) on the surface of the

catheter that encircle the CVL in the form of “sleeve” or “cover”, but do not involve the vessel wall. Despite frequent development of TDs on CVLs, data on their clinical relevance are

very limited and controversial.

Aim. To estimate the role of TDs as a risk factor for the development of DVT in children with MN. To estimate the effi cacy of antithrombotic agents in preventive care of DVT in patients

with TD.

Materials and methods. Our analysis included retrospective data from electronic case report forms on 182 CVL that were inserted to 113 patients at the age of 1–19 years old

who underwent therapy for acute lymphoblastic leukemia at FRC PHOI n.a. Dmitry Rogachev from August 2012 to December 2014. The presence of DVT and TD was estimated by

echocardiography and Doppler ultrasound study of the extracranial parts of brachiocephalic veins. Statistical analysis was conducted with the use of IBM SPSS Statistics 20 software.

Results. At least one detection of TD was registered on 63 (35 %) CVLs. The rate of TD occurrence was 2.71 per 1000 catheter-days. Odds-ratio for DVT development in patients with

previous TD was 2.75 (95 % CI 1.32–5.74; P = 0.003). The effi cacy of antithrombotic agents for the elimination of TD was assessed on the sample of 26 CVLs for which data on the

delivery/absence of therapy and sizes of TD over time were available. In case of antithrombotic agents administration (low-molecular heparins, therapeutic doses) positive dynamics

was registered in 7 (41 %) out of the 17 cases, without therapy – in 4 (44 %) out of the 9 cases. Statistically signifi cant relation between the use of antithrombotic agents and the

resolution of TD was not found (P = 0.598). The analysis of the infl uence of antithrombotic prophylaxis on the risk of DVT development in patients with TD revealed that in a group

with antithrombotic agents administration DVT was detected in 15 (48%) out of the 31 CVLs, and in a group without antithrombotic agents administration DVT was detected in 9

(32 %) out of the 28 cases (P = 0.908).

Conclusion. TDs are a risk factor for the development of CVL-associated DVT; however, the effi cacy of antithrombotic agents for prevention of TD and DVT in patients with TD is

doubtful.

A B S T R A C T N O . : P - 1 8 0

Hypercoagulation phenomenon in children with deep venous thrombosis

in the setting of treatment of malignant neoplasms

P.A. Zharkov, M.A. Gracheva, E.N. Seregina, A.V. Poletaev, A.V. Pshonkin

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: thrombosis, DVT, cancer, hypercoaguable state, thrombodynamics, clotting

Introduction. State of hypercoagulation is a laboratory phenomenon demonstrating a tendency to acceleration of thrombosis in vitro. Deep venous thrombosis (DVT) is a quite

frequent comorbidity in children with solid tumours (ST), however, the relation of this complication with the state of hypercoagulation in children is not well studied.

Aim. To estimate the presence of hypercoagulation state in children with newly diagnosed DVT in the setting of treatment of SD.

Materials and methods. The investigation included 27 children undergoing treatment for SD at the age of 2–16 years old who were newly diagnosed with DVT based on the fi ndings

of visualization (duplex sonography/computed tomography/magnetic resonance tomography/angiography). The distribution of the patients according to nosology: neuroblastoma – 7 (26 %),

sarcomas – 8 (30 %), central nervous system tumours – 1 (19 %), hepatoblastoma – 2 (7 %), other tumours – 5 (19 %) детей. Before the start of anticoagulation therapy, D-dimer

concentrations were estimated (in 22 children) as well as a global hemostatic coagulation assay – Thrombodynamics – was performed. D-dimer values over 243 ng/ml and the

enhancement of the velocity of clot growth (Vst) of more than 30 mcm/min according to the results of Thrombodynamiсs were taken as indicators of the presence of hypercoagulation.

Results. The presence of one or another indicator of coagulation was registered in 20 (91 %) out of the 22 patients, and the absence of these indicators was found in only 2 (9 %)

out of the 22 patients. The increase in D-dimer concentration was detected in 17 (77 %) out of the 22 patients, and the increase in Vst – in 17 (63 %) out of the 27 examined patients.

Given that, the increase in D-dimer concentrations was isolated in 5 (23 %) out of the 22 patients, and the isolated increase in Vst was found in 3 (14 %) children. A combination of

increased D-dimer concentration with the increase in Vst was registered in 12 (55%) out of the 22 patients.

Conclusion. One or another markers of hypercoagulation are detected in 91 % of children with DVT in the setting of treatment for ST, at the same time, the combination of increased

D-dimer concentration with the increase in Vst is registered in 55 % of children. Further investigations are required in order to estimate the prognostic value of the detection of

hypercoagulation markers in children with ST.

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A B S T R A C T N O . : O P - 2 1 3

Time-to-antibiotic administration in children with febrile neutropenia:

a quality of care initiative

Namrata Todurkar, Amita Trehan, Deepak Bansal

Postgraduate Institute of Medical Education & Research, Chandigarh, India

Key words: time to antibiotics, febrile neutropenia

Introduction. Febrile neutropenia (FN) is on oncological emergency needing admission with prompt administration of antibiotics with time to antibiotics (TTA) being a quality of

care measure.

Aim. To estimate the TTA in FN patients & evaluate causes for delay.

Materials and methods. Prospective study (July 2014 – June 2015) of patients admitted for FN. Transplant, relapsed & those presenting with hemodynamic instability were

excluded. Primary outcome: patients who received antibiotics within 60 minutes of triage assessment (TTA). Age, time of day, weekday, level of doctor performing assessment, area

of assessment & focus of infection were evaluated as predictor variables. A root cause analysis was done in cases of delayed TTA (DTTA).

Results. 211 children, age 6 years (0.6–15), with FN were evaluated. Median TTA: 60 minutes (IQR: 30–120). 78 % were examined by a senior physician (Registrar/Consultant),

28: intermediate level (3rd–6th semester residents) 18 were seen by junior physician (1st/2nd semester residents). 120/211 were assessed during working hours, 59 in the evening

and 32 at night. 105/211 evaluated in day care, 69: ward & 37 in clinic. 92 children were below 5 years. 65 % & 79 % received antibiotics within 1& 2 hours. Evaluating the predictor

variables, odds of DTTA were 2.11 (P = ns) when assessed by a senior physician. Odds of DTTA were 1.37 & 1.02 (P = ns) when assessed during working hours and weekend. Odds were

1.37 (P = ns) when assessed in clinic/day care compared to ward. Age less than 5 years had an OR of 2.0 for DTTA. (P = 0.018) DTTA odds were 1.72 (P = ns) in cases with no focus of

infection vs. those with a focus of infection. There was no correlation between DTTA and duration of admission /mortality.

On doing a root cause analysis, waiting for blood results (29 %), delay in preparing antibiotics (32 %) & time taken for getting a fi le and allotment of bed (45 %) were the major

causes of delay.

Conclusion. 65 % children got antibiotics within 1 hour of triage. Senior physician examination resulted in delay, possibly as they follow clinical judgement more than a junior

resident following instructions for FN. Delay (P = ns) was seen during working hours & day care assessment, which could be attributed to a busy day care with other jobs in the

daytime taking precedence. Weekend delay (P = ns) could be accounted for by less medical staff being on duty. Delay in children less than 5 years, which was signifi cant, was diffi cult

to rationalize. Time to complete admission formalities was the major root cause for delay accounting for 45 % of delays. No comment on prognostic signifi cance of a prolonged TTA

in pediatric FN cases can be made.

A B S T R A C T N O . : P P - 2 1 4

Evaluating the eff ectiveness of ketamine plus atropine as anaesthesia for intrathecal

chemotherapy and bone marrow aspiration at hospital, Vietnam

Kim Hoa Thi Nguyen

Hue Central Hospital, Vietnam

Key words: ketamine, atropine

Introduction. Ketamine is a phencyclidine and cyclohexamine derivative. It is unique among the sedative analgesics in producing a dissociative state between the thalamus and the

limbic system characterized by four features: sedation, analgesia, amnaesia and catalepsy. Ketamine and atropine have increasingly been used in recent years as an eff ective form of

deep sedation/anaesthesia in children in developed countries, but not in developing countries like Vietnam.

Aim. This pioneer trial aimed to evaluate the eff ectiveness of using ketamine plus atropine as anaesthetic agents for paediatric oncology procedures. From this study, we establish

a protocol for anaesthesia in paediatric oncology procedures.

Materials and methods. A descriptive and prospective study on 52 paediatric patients of both sexes (33 males and 19 females) aged 7 months to 14 years (median age: 4.5 ± 3.2

years) and with body weight between 4.5 to 40 kg (median weight: 15.3 ± 6.2 kg) was carried out from January 2015 to January 2016. The patients had been diagnosed with either

acute lymphoblastic leukaemia or acute myeloid leukaemia. They underwent intrathecal chemotherapy and bone marrow aspirations for diagnostic as well as therapeutic purposes.

After obtaining informed consent from their parents, the research was performed. Datas were analysed by Medcalc software.

Results. The total number of procedures was 127. Bone marrow aspiration was performed 59 times and intrathecal chemotherapy given 68 times. All procedures were successfully

completed. The dose of ketamine and atropine used 1.55 ± 0.31 mg/kg and 0.100 ± 0.029 mg respectively. The time taken for anaesthesia to wear off was short: 9.7 ± 9.5 minutes.

Only 0.8 % experienced apnoea; 3.1 % convulsion; 1.6 % nystagmus, and hyperactivity; 2.4 % excess salivation, and dreaming; 7.1 % vomiting; none of the patients had laryngospasm

or transient rash. All of the patients’ parents were satisfi ed with the use of anaesthetics.

Conclusion. This is a pioneer trial for children in Vietnam. 1.5 mg/kg intravenous ketamine and 0.1 mg atropine were found to be eff ective and suitable dose in children requiring

deep sedation for painful procedures and produce only minimal side eff ects. We established a protocol with the above doses and continue to apply this in order to reduce pain, trauma,

and complications and to practice safely.

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A B S T R A C T N O . : O P - 2 2 3

Features of enteral nutrition and anorexia overcoming

in hematopoietic stem cell transplantation in children

M. Kucher1, O. Pirogova1, O. Goloshchapov1, V. Karev2, A. Schvetsov1, B. Afanasyev1 1Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical

University of St. Petersburg, Russia; 2Research institute for children’s diseases, Saint-Petersburg, Russia

Key words: hematopoietic stem cell transplantation, nutrition support, anorexia

Introduction. Hematopoietic stem cell transplantation (HSCT) is a modern and eff ective method of treatment for oncological, hematological and inherited diseases. It’s associated with many

complications, among others special place take malnutrition which is formed as a result of anorexia, mucositis, “graft versus host disease” (GvHD) and reduces the eff ectiveness of treatment.

Aim. To evaluate the eff ectiveness of enteral feeding and appetite stimulants in pediatric patients with HSCT.

Materials and methods. We analyze 143 cases (40 of them were secondary) of clinical nutritionist consultations for patients with diff erent types of chemotherapy and HSCT in 2015.

The age of patients ranged from 4 months to 21 years (median 11.6 years). 109 patients underwent HSCT: allogeneic unrelative (n = 55), allogeneic relative (n = 13), autologous

(n = 4), haploidentical (n = 37); chemotherapy (n = 30); immunotherapy (n = 3). For the assessment of nutritional status anthropometry, biochemical markers were applied.

To assess the severity of GvHD – histological examination of biopsy specimens of the stomach and intestine was performed.

Results. The main syndromes were: lack of body mass of 75.5 % (n = 108), anorexia 63,6 % (n = 91), intestinal dyspepsia of 25.2 % (n = 36), malabsorption of 14.7 % (n = 21).

The greatest negative impact on nutritional status had intestinal GVHD (P = 0.0001). For prevention and correction of malnutrition, depending on type of HSCT, time after treatment

and the extent of damage of gastrointestinal tract, patients received various types of nutritional support (NS): low-microbial diet (n = 51), sipping/enteral nutrition (n = 67),

combination of methods with parenteral nutrition (n = 25). Primary NS was ineff ective in 60.8 % of cases – further decrease in anthropometric parameters and biochemical markers

of nutritional status were observed. To correct malnutrition second line NS therapy was provided (n = 40), which includes appetite stimulants, complex enzymes of the digestive

system, change of enteral nutrition regimens from polymeric mixtures to semielemental and specialized depending on the clinical situation. Second-line therapy was more eff ective:

“megestrol acetate + diet” in 72.3 % of cases; “megestrol acetate + Pediasure” in 65.3 % of cases. In the case of malabsorption syndrome greatest effi cacy and compliance – 31.5 %

of cases were noted in “megestrol acetate + Peptamen” group.

Conclusion. Patients with HSCT are at high risk to develop malnutrition due to severe violations of digestion process, anorexia, hypercatabolism in the case of GvHD. Correction

of malnutrition is complicated by low tolerance for enteral nutrition formulas. Higher effi cacy was observed when using specialized semielemental mixtures in combination with

appetite stimulants (megestrol acetate) and enzymes compared to the standard schemes of NS.

A B S T R A C T N O . : P P - 2 3 0

Patient-controlled analgesia with tramadol in children and adolescents

with gastro-intestinal mucositis after high-dose chemotherapy with autologous

hematopoietic stem cell transplantation

E. Goncharova, A. Kozlov, A. Potemkin, L. Zubarovskaya, B. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology,Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia

Key words: pain, PCA, mucositis, tramadol

Introduction. Gastro-intestinal acute mucositis (GI AM) is one of the most frequent complications after high-dose chemotherapy with autologous hematopoietic stem cell

transplantation (HDCT with auto-HSCT). The incidence of GI AM varies from 75 to 99 % (L. Vagliano et al., 2011). Typical feature of GI AM is a various intensity pain syndrome. According

to European Pain Federation EFIC recommendations patient-controlled analgesia (PCA) was used in the study.

Tramadol is widely administered in Raisa Gorbacheva Memorial Institute of Children Oncology Hematology and Transplantation, First Pavlov Saint Petersburg State Medical University

for purposes of pain control. Tramadol’s metabolite - mono-0-desmethyltramadol demonstrates mechanisms of opioid and non-opioid actions and relatively prolonged elimination

half-life. The role of tramadol administration in children is not completely established but there are data supporting tramadol as moderate pain reliever with high effi ciency and safety

profi le (WHO). The feasibility of tramadol administration by means of PCA also needs further investigation.

Aim. Evaluation of effi ciency and safety of PCA with tramadol in children and adolescents with GI AM after HDCT with auto-HSCT.

Materials and methods. In the study 33 patients after HDCT with auto-HSCT were included. Median age was 9 (range 3–18). Indications for HDCT with auto-HSCT were

neuroblastoma (n = 14), Ewing’s sarcoma (n = 8), medulloblastoma (n = 7), Hodgkin’s lymphoma (n = 2), ganglioneuroblastoma (n = 1), primitive neuroectodermal tumor (n = 1).

All patients had GI AM grade 2–3. Duration of cytopenia varied from 7 to 20 days. Median length of pain reliever administration was 7 days (range 3–12).

In order to analyze pain intensity Face Scale and Visual Analogue Scale (VAS) were used. Patient-controlled anelgesia was administered in all patients (loading dose 0.5 mg/kg,

background infusion 0.25 mg/kg, bolus 0.25 mg/kg, lockout 15 minutes).

Results. Before the start of PCA median pain intensity (VAS score) was 7 (range 5–10). The median of pain score, patients could cope with was 4 (range 2–5). The median number of

pain-associated night awakenings was 3 (range 1–5). The median VAS score after the start of pain relief therapy was 3 (range 0–4) and the most of children didn’t wake up at night

or woke up only once. Only in 2 patients (6 %) adequate level of analgesia couldn’t be achieved and morphine was administered as a next drug in WHO pain relief ladder. Adverse

eff ects of tramadol that were registered in the study included drowsiness in 6 patients during fi rst two days (didn’t require correction), nausea in 3 (9 %) patients (required antiemetic

therapy), hearing hallucinations in 1 (3 %) patient with medulloblastoma (required withdrawal of tramadol and switch to morphine).

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Conclusion. PCA with tramadol is eff ective and safety pain reliever in children and adolescents with GI AM grade 2–3 after HDCT with auto-HSCT. Low incidence of night breakthrough

pain episodes is associated with prolonged elimination half-life.

A B S T R A C T N O . : O P - 2 4 6

Spectrum of microbiological infections and resistance pattern in pediatric oncology patients:

experience from a tertiary care center in North India

Vinod Gunasekaran, Nita Radhakrishnan, Veronique Dinand, Shiny Joy, Chand Wattal, Anupam Sachdeva

Sir Ganga Ram HospitaL, India

Key words: pediatric oncology, microbiological infections, antibiotic resistance

Introduction. In developing countries, infections pose the major challenge in curing pediatric malignancies. Data on spectrum of infections and resistance patterns in pediatric

oncology from developing countries is sparse.

Aim. The aim of our study is to analyze the spectrum of microbiological infections and their resistance patterns among children receiving chemotherapy presenting with fever.

Materials and methods. Pediatric oncology patients presenting with fever were enrolled (2007–2015). Results of the microbiological investigations were recorded. The spectrum

of infections and their sensitivity pattern were analyzed.

Results. There were 1023 episodes of fever evaluated. 590 (57.6 %) episodes were febrile neutropenia. 225 (22 %) had microbiologically documented infections. Since 2012, around

90 % patients had central venous access devices. Gram positive, gram negative, fungal, viral, mycobacterial and parasitic infections accounted for 53 (23.5 %), 89 (39.5 %), 51 (22.6 %),

30 (13.3 %), 1 (0.4 %) and 1 (0.4 %) cases respectively. Spectrum of gram positive infections (n = 53) included coagulase negative Staphylococcus (65.7 %), Staphylococcus aureus (11.3

%), Enterococcus fecium (13.2 %), Enterococcus fecalis (3.7 %), Streptococcus pneumoniae (1.8 %) and other Streptococcal species (4.3 %). Gram negative infections (n = 89) included

Klebsiella pneumoniae (29.2 %), Pseudomonas species (21.7 %), Escherichia coli (16.8 %), Acinetobacter species (8.9 %), Stenotrophomonas maltophilia (5.6 %), Proteus mirabilis (2 %),

Salmonella species (2 %), Nocardia (1 %), Chryseobacterium (1 %), Elizabethkingia meningoseptica (1 %) and unidentifi ed (in 11 %). Invasive aspergillosis was diagnosed in 34 cases

(using galactomannan assay). Fungus isolated from body fl uids (n = 17) included Candida albicans (5), Candida tropicalis (3), Candida parapsilosis (3), Candida glabrata (1), Candida

haemulonii (1), Candida pelliculosa (1), Aspergilllus (1) and Trichosporon asahii (2). Documented viral infections included Cytomegalovirus (19), Dengue (6), Epstein–Barr virus (4)

and Herpes simplex virus (1). One child had Cryptosporidiosis. With advancing years, incidence of gram positive infections decreased. Vancomycin resistance has not been observed

among gram positives. Among Klebsiella and E.coli, extended spectrum beta lactamases were produced by 96 % and 81 % respectively, whereas carbapenemases were produced by

44 % and 31 % respectively.

Conclusion. Gram negative bacteria are the major isolates at our center. Gram positive isolates have shown reduction with time due to better nursing care of central lines. Multidrug

resistant gram negative bacteria and aspergillus infections are emerging concerns.

A A B S T R A C T N O . : P P - 2 6 4

Successful emergency helicopter transfer for a boy of mediastinal lymphoblastic lymphoma

complicated with reexpantion pulmonary edema and cardiac tamponade

Ritsuo Nishiuchi, Naoko Oura, Chiho Tokorodani, Kiyoshi Sasaki

Kochi Health Sciences Center

Key words: helicopter transfer, reexpantion pulmonary edema, cardiac tamponade

Introduction. Pleural and pericardial eff usions are common oncologic emergencies, while reexpantion pulmonary edema (REPE) is a rare and potentially lethal complication for

removal of pleural eff usion.

Aim. Case repot.

Materials and methods. Case report.

Results. An eleven-year-old boy presented at a local hospital complaining of cough for the prior two weeks and appetite loss. A chest X-ray and computed tomography (CT) showed

fi ndings of a large right pleural eff usion and anterior mediastinal mass. Thoracentesis was performed resulting in the aspiration of 1900 ml. A few hours later, he started coughing,

had dyspnea and pulse oximeter reading of 79 %. After removal of additional 700 ml eff usion, his condition deteriorated and tracheal intubation was performed. The emergency

helicopter transfer was requested at 17:30. He was picked up at 18:02 and arrived in our hospital at 18:53. Vital signs were: temperature of 38.0 °C pulse of 153/min, blood pressure

of 145/93 mm Hg, respiratory rate of 40/min, a pulse oximeter reading of 82 % with 10 L/min oxygen. CT showed diff use area of consolidation and ground-glass opacity in the right

lung, suggestive of REPE. Echocardiography showed cardiac tamponade caused by pericardial eff usion and compression by the tumor. Surgical drainage of pericardial eff usion and

biopsy of mediastinal mass with right anterolateral thoracotomy was performed from 3:32 to 7:11 on the 2nd

day of admission. Simultaneously prednisolone was started for treatment

of lymphoma. After surgery, his oxygen saturation and hemodynamics improved gradually. A diagnosis of T-cell lymphoblastic lymphoma (CD2+, CD3+, CD4+,CD 7+, CD8+, CD10+) was

made based on histopathology of biopsy and fl ow cytometry results of pleural eff usion. On the 4th day, his tracheal and pleural drainage, pericardial drainage tube were removed. On

the 9th day, he started intensive chemotherapy according to JPLSG ALB-NHL03 protocol. He completed chemotherapy and remains disease free.

Conclusion. Pediatric oncologists must recognize oncologic emergencies and treat them quickly with cooperation of emergency physicians and surgeons.

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A B S T R A C T N O . : O P - 2 8 8

Indicators of the Russian infrastructure of inpatient pediatric palliative care with malignancies

D.V. Nevzorova1, E.V. Polevichenko2, L.S. Kochetkova3

1Hospice № 1 named after V.V. Millionschikova, Moscow, Russia; 2Pirogov Russian National Research Medical University, Moscow, Russia; 3Research institution – Higher School of Economics, Moscow, Russia

Key words: children, pediatric palliative care, malignant diseases

Introduction. Intensive development of infrastructure of palliative medical care (PMC) for children in Russia is focused on the indicator value of the Government program of

the Russian Federation “Healthcare development” – the availability of hospital beds for providing PMC (up to 2.08 per 100 thousand children by 2020). Nosological structure of

patients who these beds are provided for during hospitalization considerably in diff erent subjects of the Russian Federation and refl ects healthcare quality indicators including

compliance with clinical guidelines and pain management standards.

In 2013 998 children with malignant neoplasms (MN) under 18 years died in the Russian Federation. In order to plan and improve pediatric oncological care quality in regions

it is necessary to evaluate the availability of stationary palliative medical care (PMC) for children with malignant neoplasms (MN).

Aim. To evaluate the usage of bed capacity of PMC for children of the Russian Federation for incurable patients with MN in 2014–2015.

Materials and methods. A retrospective analysis of reported data provided by the governmental authorities in the fi eld of health protection of the subjects of the Russian Federation.

Results. As on October 1, 2015 PMC for incurable children in the RF was provided by 5 pediatric hospices, 29 inpatient departments of PMC for children and 20 mobile patronage

teams of PMC for children.

Total bed capacity of PMC for children was 566 that ensured treatment of 2778 children over 9 months in 2015. Children with MN (n = 388) comprised 14.0 % of the total number of

children who received care on palliative beds while the numerically prevalent nosological group during inpatient PMC consisted of children with neurologic pathology (n = 1755; 63.2 %).

Similar fi gures in 2014 were the following: total number of patients who received treatment using beds of PMC for children was 2947, 382 (13.0 %) of which had MN.

Incurable children with MN received care on palliative beds not in all subjects of the RF. Only 29 (34.1 %) of 85 subjects of the RF admitted these patients in order to provide stationary

PMC. On average the inpatient PMC was provided to 13.4 of children with oncological diseases per 1 subject of 29 mentioned over 9 months. No signifi cant changes were noted

in comparison with the similar data of 2014 when in 23 subjects the inpatient PMC was provided to 382 children with MN (on average 16.6 children per 1 subject).

Conclusion. In 2014–2015 in the RF children with oncological diseases comprised 12–13 % of all patients who received treatment using beds for stationary PMC that corresponds

to the data from international medical statistics.

Not more than in one third of all subjects palliative beds were used for children with MN. No more than one third of the deceased children with MN received stationary PMC.

Taking into account undeveloped network of ambulatory forms of palliative care for children and the absence of pediatric non-invasive forms of narcotic analgesics it is necessary

to improve availability of the stationary PMC for children with oncological diseases in those regions of Russia where it was not established and was not used in 2014–2015.

A B S T R A C T N O . : O P - 3 0 0

Cytomegalovirus (CMV) viraemia and disease in children with haematological malignancies

undergoing conventional chemotherapy: a study from a referral cancer centre in India

Anand Koratagere Chandrashekhar, Tushar Idhate, Gaurav Narula, Brijesh Arora, Shripad Banavali

Tata Memorial Hospital, Mumbai, India

Key words: CMV infection, children, non-transplant setting

Introduction. There is a paucity of data on Cytomegalovirus (CMV) infection in children with haematological malignancies in non-transplant settings, which is critical to plan timely

therapy and reduce morbidity and mortality.

Aim. To study the clinic- biological features of CMV infection in children with hematological malignancies receiving myelo-suppressive chemotherapy in non- transplant setting.

Materials and methods. Between January 2008 and November 2015, we screened all children with haemato-lymphoid malignancies having unexplained fever, prolonged cytopaenia,

haemophagocytic lymphohistiocytosis (HLH) or clinical CMV disease, for CMV using RT-PCR technique. CMV-positive episodes were analysed for clinical features and response to treatment.

Results. Seventy children had CMV infection. Of them, 61 (87 %) had CMV DNAemia and 9 (13 %) had CMV disease. The primary diagnoses were ALL (n = 59, 85 %), AML (n = 2),

NHL (n = 6), Hodgkin’s lymphoma (HL, n = 2), and CML (n = 1). The median age was 12 years. For ALL patients, CMV DNAemia and disease occurred mostly during either consolidation

(46 %) or maintenance (36 %) phase. The most common presenting feature of CMV infection was unexplained fever without focus (n = 68, 97 %). Of these, 37 (54.5 %) had no

associated cytopaenias. All became afebrile at median ganciclovir duration of 3 days (range, 2–7 days). The rest (n = 31, 45.5 %) had unexplained fever with cytopaenia, and all

recovered after a variable period of 3 to 28 days (median, 15 days). Two of them developed HLH and were treated with HLH protocol along with antiviral therapy. For patients with

CMV viraemia, CMV titres became undetectable after a median period of 17 days (range 7–34 days). Five had relapse of CMV-viraemia: two had complete response and survived,

while 3 died of primary malignancy during CMV therapy.

For the 9 with CMV disease, their primary diagnoses were ALL (n = 6), AML (n = 1), HL (n = 1), and Burkitts lymphoma (n = 1). Disease sites were chorio-retinitis (n = 2), pneumonitis

(n = 3), gastrointestinal disease (n = 2) and encephalitis (n = 1). In addition, one patient had both GI infection and CMV encephalitis. After treatment with I.V. ganciclovir, 5 patients

recovered fully, 2 died of CMV, one died of progressive primary disease and one was lost to follow up. Out of the 2 children with chorio-retinitis, one had permanent visual defi cits

while the other recovered normal vision.

Conclusion. CMV DNAemia and disease are prevalent in children with haematological malignancies on standard-dose therapy. ALL patients were more commonly aff ected, usually

during the consolidation phase of ALL therapy. Most patients presented with unexplained fever with or without prolonged cytopaenias. Approximately 15 % can progress to HLH or

CMV disease if not detected in time. Timely detection and therapy with ganciclovir is curative in most children.

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A B S T R A C T N O . : O P - 3 5 2

Disparities in the global landscape of palliative care clinical trialsCatherine Lam1, Scott Howard2

1St. Jude Childrens Research Hospital; 2University of Memphis

Key words: clinical trials, palliative care, global health, disparities

Introduction. Globally, palliative care is under-developed in many regions, as highlighted by the fi rst World Health Organization (WHO) and Worldwide Palliative Care Alliance

(WPCA) Global Atlas of Palliative Care at the End of Life (2014). Understanding the landscape of research across settings globally can identify key implementation gaps.

Aim. We compared palliative care clinical trials across regions and income-settings relative to the expected patient needs as defi ned by the WHO/WPCA Atlas, and evaluated

the current extent of inter-regional research.

Materials and methods. The WHO International Clinical Trials Registry Platform (ICTRP) was searched for palliative care trials (interventions, conditions, and/or key words),

including data from 16 international sources across all six WHO regions (Americas (AMRO), African (AFRO), Eastern Mediterranean (EMRO), South East Asia (SEARO), and Western

Pacifi c (WPRO)), last searched 1/29/2016. Data was extracted for trials pertaining to children (age 0–14 years) and adults. Primary outcome was proportion of trials conducted in

countries per WHO region and World Bank income group (low-income [LIC], lower-middle-income [LMIC], upper-middle-income [UMIC], and high-income [HIC]). Comparative data

was extracted from the WHO/WPCA Atlas. Secondary outcomes included the countries and regions represented per trial.

Results. Across 781 palliative trials registered, 58 countries were represented. Of the 746 adult trials, there were disparities in the proportion of trials from regions relative to patients

in need, including under-representation in SEARO (5 % of trials vs 22 % of burden) and over-representation in EURO (44 % vs 22 %). There was under-representation in the proportion

of trials including LMIC (5 % vs 29 %) or UMIC (7 % vs 41 %), and disproportionately more including HIC (90 % vs 22 %). No registered trial specifi ed inclusion of a low-income country.

Overall, although low- and middle-income countries account for more than 3/4 of the palliative care burden, they are represented by less than 1/8 of the trials, such that patients in

HIC have access to 27 times more trials per patient in need compared to those outside HIC. The 35 (4 % of total) trials inclusive of pediatric needs appeared to correspond to WPCA’s

estimate of this age group refl ecting 6 % of global needs. However, comparing proportion of trials to burden by region, there was under-representation in AFRO (0 % of trials vs

49 % of global burden), SEARO (0 % vs 24 %), and EMRO (3 % vs 12 %), with over-representation in other regions, notably AMRO (66 % vs 5 %). There was under-representation from

LIC (0 % vs 35 %), LMIC (3 % vs 49 %), UMIC (6 % vs 14 %), and over-representation from HIC (94 % vs 2 %). Although low- and middle-income countries have 98 % of children with

palliative care needs, they are represented by only 9 % of the trials, such that patients in HIC have access to 523 times more trials per patient in need. Across all trials, most (93 %) were

single-country studies; of the 53 multi-country trials (involving up to 16 countries, 6 regions and 3 income groups), most involved countries within one region (60 %) and one income

group (72 %). Only 3 pediatric studies (9 %) were multi-country trials.

Conclusion. Disparities in palliative care clinical trials include under-representation of trials relative to needs in South-East Asia and low- and middle-income countries for both adults

and children. To address implementation gaps in these areas, further research investment and inter-regional collaborations are warranted.

A B S T R A C T N O . : O P - 3 6 3

Evaluation of chemotherapy-induced oral mucositis in pediatric oncology hospital

Sarah Mohamed Abdelsamie, Mohamed Abdelrahman, Doaa Abdelmegeed

Children’s Cancer Hospital 57357, EgyptKey words: mucositis, pediatric oncology

Introduction. Oral mucositis (OM) is a common side eff ect experienced during chemotherapy course, and it can have a signifi cant impact on the quality of life of patients.

The incidence of oral mucositis in pediatric cancer patients was evaluated in 105 children with cancer, as well as associated risk factors, and patient adherence through interviewing

patients/patient relatives and reviewing data from patients medical records.

Aim. To demonstrate the incidence of chemotherapy-induced oral mucositis (OM) in pediatric oncology hospital, identify patient populations at highest risk, evaluate level of patient

education concerning mucositis, evaluate patient awareness and adherence to OM prophylaxis counseling.

Materials and methods. 105 chemotherapy patients/patient relatives were interviewed to report any experience on oral mucositis since therapy started, and to mention the mouth

care instructions they know. At the same time, the 105 patients’ medical records (MR) were reviewed to gather demographics and related information.

Results. Overall incidence of mucositis was 79 % (n = 83). A total of 79 % (n = 83) of patients developed OM at least once with a total no. of 163 OM events with 1.96 (≈2) OM

events developed per patient. Confl icts between patient answers and patient Medical Records documentation were faced (36 % of 105 patients) due to either inappropriate OM event

documentation (35 % of total 83 positive OM patients) or patient unawareness of the event (9 % of total 105 patients). Regarding gender; females were at greater risk of developing

OM than males with (85 %) developed OM compared to (79 %) within males. Greater child age was a risk for increased susceptibility to OM with 92 %, 81 %, 72 % within the studied

age ranges (0.2–6), (7–12), (13–19) years, respectively. Oncology Diagnosis and disease stage were important factors that within AML, NHL, OS, RMS, ALL patients, OM developed

in 100 %, 87 %, 78 %, 75 %, 72 % of each category, respectively with 100 % OM within relapsed patients compared to 77 % within fi rst line therapy patients. Incidence of OM per

patient diff ered among diff erent protocol stages with 3.67, 1.8, 1.53, 1.41, 1.25, 1.17, 1,1 event per patient for pre/post operative chemotherapy, re-induction, fi rst line induction,

consolidation, second line therapy, continuation, intensifi cation and re-intensifi cation, respectively. Severity of OM was described in 70 % of total 163 OM events either from patient

MR or patient interview answers while 30 % of the events could not be obtained neither from patient MR nor patient interview answers, with grades 1, 2, 3, 4 contributed to 21 %,

32 %, 12 %, 5 %, respectively. Again inappropriate documentation of OM event was faced that grades for 56 % OM events (of total 163 events) were not documented on patients

MR. While 100 %, 74 % of grades 4, 3 events were appropriately documented, only 62 %, 51 % of grades 2, 1 were appropriately documented, respectively. Regarding prophylaxis

medications usage, 89 % of 105 patients used prophylaxis (Chlorhexidine, Nystatin, Miconazole), but only 29 % exhibited adherence to appropriate use. Non-adherence was due to

either unacceptable product taste or patient unawareness.

Conclusion. Increased patients susceptibility to chemotherapy-induced OM was observed. Patient gender, age, diagnosis, protocol stage and disease stage were contributing factors

to OM. Level of event documentation and patient education was sub-optimal.

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A B S T R A C T N O . : O - 3 8 8

Percutaneous endoscopic gastroscopy in children with cancer and organic CNS diseaseE.A. Dogadov, S.B. Bondarenko, P.V. Shumilov, S.V. Kulikov

Applied Research Center for Specialized Medical Care of Children named after V.F. Voyno-Yasenetsky, Russia

Key words: enteral feeding, gastrostomy, children’s oncology, CNS tumors, palliative care

Introduction. Optimal nutritive status in children with CNS tumors is of particular importance. Nasogastric tube and standard surgical gastrostomy are conventional methods

to provide longitudinal nutrition. Prolonged use of nasogastric tube negatively impacts patient’s quality of life, impairs daily care and may lead to complications. Percutaneous

gastrostomy may improve patient’s quality of life.

Aim. Improvement of quality of life in children with cancer and organic CNS disease by choosing an optimal method of gastrostomy.

Materials and methods. From September 2012 to February 2016 eighty three endoscopy-assisted gastrostomies were performed in children aged 5 month to 17 years with CNS

tumors and organic CNS disease. Pull method was used in 49 cases and Russell method in 34 cases. In 2 cases standard procedure was modifi ed with laparoscopy due to left liver lobe

enlargement and severe scoliosis aff ecting gastric topography.

Results. Pull method is less invasive and of 10–15 minute duration. There were no surgical complications, but intermittent leakage of gastric content around the tube complicating

post-surgical care was observed in all patients. Percutaneous gastrostomy using Russell method provided a tight sealing between the tube, the stomach and abdominal wall

preventing gastric content leakage and infl ammation of the adjacent tissues. In 2 cases standard procedure was modifi ed with laparoscopy due to left liver lobe enlargement

and severe scoliosis aff ecting gastric topography. Gastrostomy in these patients was placed in non-typical areas. No complications were observed.

Conclusion. Both methods of percutaneous gastrostomy used in our study provided conditions for full enteral feeding but Russell method was associated with better sealing of

the gastrostomy canal, facilitated care and better quality of life. In case of atypical topography of the stomach percutaneous gastrostomy should be supplemented by laparoscopy

procedures.

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A B S T R A C T N O . : P - 1 2 9

The organization of the social help to the families having a child with an oncological disease

at the Medical and Rehabilitation Scientifi c Center “Russian Field”

M.Ye. Kokoreva, G.Ya. Tseitlin, A.F. Karelin, N.N. Volodin

Medical and Rehabilitation Scientifi c Centre “Russian Field” of the Federal Research Centre of Pediatric Hematology,Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia

Key words: sibling, disabled child, medico-social work

Introduction. A child’s grave disease, long-term treatment, a reluctant isolation, exclusion from the customary way of life, a change in the system of values, the uncertainty of

the nearest and the distant future all constitute a severe stress for the whole family with a child suff ering from an oncological disease. The life of the majority of such families in

modern Russia is characterized by a number of serious social problems. A family fi nds itself in socio-psychological isolation, having no moral or fi nancial support either from the close

circle of friends or from the government. Siblings, i.e. brothers and sisters of an ill child, end up in a diffi cult situation since all moral and fi nancial resources of a family are as a rule

focused on the ill child. Our study showed that 20 % of parents get a divorce after their child goes down with a disease. The fi nancial standing of the majority of such families is poor:

75.5 % spends 50–100 % of their cumulative income on food; 27.8 % cannot purchase durable goods; for 18.7 % of respondents buying clothes presents a fi nancial diffi culty;

17.7 % of respondents struggle from paycheck to paycheck. Their home is usually not suited for a disabled child or a family does not have their own accommodation at all. The situation

is further aggravated by the complete ignorance of 90 % of parents in legal issues, especially in those concerning various aspects of social protection (privileges, welfare benefi ts, the

registration of a disability status). A family is the main element in the system of a disabled child’s socialization, education and professional orientation, which is why providing help to

a family is a necessary condition for a disabled child’s rehabilitation.

Aim. The aim of the medico-social eff orts in Pediatric Oncology is to achieve a maximal standard of health and of a psychosocial adaptation of ill children as well as to provide

a complex psycho-pedagogical and socio-legal help to a family.

Materials and methods. In the structure of the Medical and Rehabilitation Scientifi c Centre «Russian Field» there is a medico-social group organized for a social accompaniment

of ill children and their family members. The primary objectives are the following: 1) diagnosing a family’s social problems; 2) informing medical offi cers, psychologists and other

members of a multidisciplinary team about a child’s problems, the socio-psychological situation in the family etc; 3) taking measures for the social adaptation of a child and his/

her family members; 4) providing legal and informational support to children and their family members as well as providing help with the registration of a disability status, the

improvement of the living conditions etc; searching for additional fi nancial resources, providing accommodation; 5) cooperating with health, social security, education authorities etc

at place of residence in order to provide help to a family after a discharge from hospital.

Results. Thus, medico-social eff orts are characterized not only by a rehabilitational but also by a preventive orientation. The leading role in medico-social measures organization is

played by social service specialists with suffi cient knowledge in such related fi elds as Social Security, Pedagogics, Psychology and Law. The functions of a social service specialist consist

in providing legal, medical, psycho-pedagogical, fi nancial and other help as well as prompting a family to achieve fi nancial independence.

Conclusion. The experience of the Group for medico-social eff orts showed its high effi cacy in resolving families’ social problems as well as in integrating a disabled child into society.

PSYCHOSOCIAL

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A B S T R A C T N O . : O P - 1 5 4

Testing of cognitive, emotional and behaviour disorders in children

with acute lymphoblastic leukaemia

V.N. Kasatkin1, R.B. Miroshkin1, A.I. Karachunsky1, Yu.V. Rumyantseva1, Е.V. Zhukovskaya1,

S. Malykh2, V. Ismatulina2, I. Voronin2

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2Psychological Institute, Russian Academy of Education, Russia

Key words: cognitive functions, emotional and behaviour sphere, acute leukaemia

Introduction. The results of a number of studies suggest the potential negative impact of modern methods of cancer treatment (chemotherapy, radiotherapy) on cognitive

functioning of children and adolescents, however, specifi c results of certain studies are rather contradictive, in particular due to the lack of comparability of the study groups.

Aim. To evaluate the eff ectiveness of protocol of diagnostics of neuropsychological disorders in patients with acute lymphoblastic leukaemia (ALL) within the randomized pilot study.

Materials and methods. 81 patients from the Rehabilitation Centre “Russian Field” of the FRC PHOI n.a. Dmitry Rogachev with ALL who had fi nished treatment took part in the pilot

study of emotional and behaviour and cognitive functions.

According to the randomization within the protocol ALL-MB-2008 a part of the patients (37 children) received cranial irradiation with the 12 Gy total focal dose, the rest 24 boys

and 20 girls received additional intrathecal injections of chemotherapeutic agents (methotrexat, cytosar, prednisolone). A set of neuropsychological tests of the hardware-software

complex CANTABeclipse (Cambridge Cognition, Great Britain) and a T. Achenbach checklist were used for the cognitive functions assessment. All methods have high retest reliability

(from 0.73 to 0.95) (M. Luciana, 2003).

Results. The fi ndings of visual-motor coordination test, understanding of instruction showed disorders in the visual-motor sphere in research subjects.

A spatial memory test suggesting memorization of stimulation pulses in space shows low degree of this function. Pediatric patients who had not received the course of radiotherapy

have more effi cient working memory abilities compared to the patients who had received irradiation. During investigation of emotional and behaviour sphere according to the results

of the Achenbach checklist some problematic issues related to the patients’ behaviour and emotional and behaviour sphere can be clearly outlined when compared with the norm.

Almost all children show abnormalities concerning all studied behaviour patterns.

Conclusion. Combination of hardware-software complex CANTABeclipse (Cambridge Cognition, Great Britain) tests and the T. Achenbach checklist in one protocol ensures

eff ective diagnostics of neurocognitive and emotional and behavioural disorders in the patients who had ended ALL-therapy, helps to defi ne guidelines for the development and

implementation in practice of further targeted rehabilitation activities.

A B S T R A C T N O . : P - 1 7 3

Social isolation as a long-term psychological side eff ect of bone

marrow transplantation in children

S. Oleshko, O. Chernenko, O. Ushakova, D. Bakun, Yu. Fedyukova, A. Borovkova,P. Kozhokar, O. Paina, K. Ekushov, L. Zubarovskaya, B. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology,Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Russia

Key words: long-term psychological side eff ect, social isolation

Introduction. Hematopoietic-stem cell transplantation (HSCT) is one of the most advanced methods of treatment of the patients with blood and genetic disorders. However despite

its proven eff ectiveness for the main condition, allogenous transplantations have serious complications and side eff ects, which are accompanied by disturbance of physical, emotional

and psychological condition of the patient.

Aim. To study the determinants of psychological adversity in children and adolescents post-HSCT.

To create a list of guidelines which are directly suited to those determinants for a rehabilitation of the patients.

To develop a system of a timely detection of psychological and psycho-social disorders of the patients during the period of post-transplant care.

Materials and methods. The study introduces us to the results of psychological examinations of children and adolescents that have undergone a HSCT (n = 11, age 7–13 years old,

post-transplant care period 1–5 years) as well as their parents or guardians, that have accompanied them in the process of the treatment.

Diff erent kinds of methods were used, such as, but not limited to:

• Projective “Pip Wilson Test”

• Projective test “Drawing of the Family”

• Projective test “Nonexistent Animal”

• Special questionnaire for parents or guardians, aimed at detecting the sphere of possible psychological disorder of the child or teenager

• Parents’ questionnaire for assessing family functioning aimed to diagnose the style of the parenting and its’ possible dysfunctions.

Results. All the children that were the subjects of the study were being homeschooled.

64 % of the children did not participate in any after-school activities and also did not interact with their peers.

27 % of the children were spending 4–10 hours on average in front of a television set or a personal computer.

Child loss phobia was diagnosed in only 18 % of the parents. Overprotection signs were more prominent (45 %) which shows that the responsibility for an adolescent’s life planning

was shifted towards their parents. This leads to the child’s isolation, their inability to acquire social experience in any environment other than their parents’ home, which, respectively,

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Conclusion. Distinctive aspects of parenting of the children after HSCT often lead to a signifi cant limitation of their social experience and to the specifi c personality disorders. It is vital

to integrate psychological support in form of counseling about the importance of communication and interaction of a child or adolescents with their peers.

In order to form such “School for the Patient” one needs the help of certain specialists, such as

• experienced psychologists, who understand the importance of the issue and consequences of lack of action;

• doctors, who would help to defi ne the amount of social contact for each patient;

• teachers from schools attended by our patients.

In order to inform the teachers of the special needs of post-HSCT children we have written the “Guideline for the Teachers” that informs school personnel about possible psychological

and physical risks that need to be taken into account when a post-HSCT pupil is in class.

To sum it up, institution like this would provide a more complete personal, social and emotional development in the post-transplant period which will lead to improvement of the

quality of life.

A B S T R A C T N O . : O P - 1 7 7

Clinical psychological rehabilitation program of the Federal Research Center of Pediatric

Hematology, Oncology and Immunology named after Dmitry Rogachev for children with

oncological diseases

R.B. Miroshkin, E.V. Fisun, V.N. Kasatkin, N.N. Volodin, A.F. Karelin

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia

Key words: rehabilitation, cognitive functions, clinical psychological diagnosis, neuropsychological correction, oncological disease

Introduction. Lack of proven rehabilitation clinical psychological programs for pediatric cancer survivors. In the process of the development of clinical psychological programs for

pediatric cancer survivors, a question of the compatibility of medical measures universality with the specifi c character of disorders occurred during the course of disease and treatment

constantly arises.

Aim. The creation and implementation of clinical psychological rehabilitation program with due regard to diagnostic data and based on neuropsychological correction of disturbed

cognitive functions.

Materials and methods. Any corrective measures should be congruent with thorough preliminary diagnosis. Clinical psychological diagnosis performed at the Treatment

and Rehabilitation Scientifi c Centre “Russkoe pole” of FSBI “FRC PHOI n.a. Dmitry Rogachev” of the Ministry of Health of Russia includes.

1. Clinical psychological pathopsychological examination.

2. Clinical psychological neuropsychological examination .

3. A complex of automated clinical psychological diagnostic examinations of cognitive functions.

Correctional clinical psychological programs are designed with due consideration of the obtained data. They present a complex of measures aimed at neuropsychological correction of

certain dysfunctions. These include exercises improving interhemispheric connections, cortical and subcortical interactions. The correction is divided into 2 stages.

1. Correction of emotional impairments by the method of biofeedback.

2. Instrumental methods of the correction of cognitive functions. (FitLight, CogniSense, DynaVision).

3. Interactive correction of cognitive functions:

a. mechanical (Pertra®)

b. computer-based (iPad)

c. paper.

Results. The investigations of cognitive impairments in children with neurooncological diseases have revealed that the overall majority of patients (82 %) has the degradation of

intelligence, educability and refocusing; 100 % of patients have visual, motor and conceptual diffi culties of various degree; 63 % of patients have shown a decrease in visuospatial

memory span. In addition, more than 80% of subjects experience various diffi culties in the solution of spatial tasks and motor control. Those children who underwent treatment for

hematological diseases have motor and visual impairments (60 %) as well as working and spatial memory dysfunctions, defi ciencies in planning and control (75 %).

Conclusion. Thus, the combination of comprehensive diagnosis, standardized blocks of correctional exercises and their focused use depending on the nature of dysfunctions provide

us with a fl exible and eff ective tool for clinical psychological rehabilitation.

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A B S T R A C T N O . : P - 1 8 1

To the justifi cation of the investigational approach to factors of psychological adjustment

to hematopoietic stem cell transplantation in pediatric oncology/hematology

A.Ye. Khain

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: psychological adjustment, coping, distress, resilience, hematopoietic stem cell transplantation, a complex approach

Introduction. The modern organization of therapeutic process in pediatric oncology/hematology enables the participation of specialists from psychosocial services at various stages

of treatment. A preliminary psychological preparation as well as support during the treatment are especially needed in case of hematopoietic stem cell transplantation (HSCT) since

this type of therapy is unavoidably accompanied by isolation and a large number of requirements and limitations, being one of the most psychologically challenging treatments both

for the child and the family (C.M.J. Vrijmoet-Wiersma, 2009; A.F. Patenaude, 1990). However, due to the organizational and methodological issues of the conduct of psychological

studies in this fi eld, there is a certain defi ciency of evidence-based standards and recommendations for psychological support of the HSCT process in Pediatrics (A.Ye. Khain, 2015).

Aim. The analysis of approaches, limitations and relevant problems in order to further investigations of psychological adjustment to hematopoietic stem cell transplantation factors

in pediatric oncology/hematology in Russia.

Materials and methods. Data from publications covering the study of a child’s adjustment and coping to stress situations associated with threats to health and wellbeing in pediatric

oncology/hematology with the special attention to HSCT.

Results. Studies of the psychological distress of children undergoing treatment of oncological/hematological disease as well as their families have been actively developing

in the recent decades. Due to large investigational projects, a great array of data regarding distress levels, quality of life and peculiarities of coping is being accumulated

(S.L. Heather Jim, K.L. Syrjala et al., 2012; C.M.J. Vrijmoet-Wiersma, R.M. Egeler et al., 2009). At the same time, a number of authors point out a large amount of contradictions

in the obtained data, the lack of a complex approach to the study of the interrelation between various adaptation factors, the necessity of an additional consideration of sociocultural

and familial aspects of coping as well as of the perception of a disease and its treatment (S. Phipps, 2004; A.F. Patenaude, M.J. Kupst, 2005). This problem is associated both with the

diffi culties in controlling a large number of factors, the lack of existing assessment methods and with insuffi ciently clarifi ed criteria of adaptation success not only in the personal

but also in the familial perspective as well as in respect to various kinds and stages of treatment. The Russian medical psychology places great emphasis on the development of

a complex approach to the consideration of factors of personal and familial stress adaptation (A.B. Kholmogorova, 2002; N.A. Sirota, 1994; E.V. Kyftyak, 2010). Still, there are relatively

few investigations involving children undergoing treatment of oncological/hematological diseases in the Russian medical centers. There are no studies in Russia on peculiarities

of psychological adjustment to such a modern and specifi c treatment as HSCT.

Conclusion. The necessity of the further development and implementation of psychological service standards in Pediatric Hematology/Oncology gives a new perspective on the issue

of evidence-based approaches to psychological interventions and support (L. Wiener, A.E. Kazak et al., 2015). Despite the large amount of accumulated data, further eff orts are needed

in analyzing the interrelation between the factors of personal and familial adjustment to various kinds of treatment. The investigation not only of maladaptation risk factors but also of

resilience (S. Phipps), the consideration of systemic factors (the familial system, sociocultural peculiarities of perception) are needed to understand criteria of successful psychological

adaptation and to develop psychosocial care programs for children undergoing HSCT and their families.

A B S T R A C T N O . : P - 1 8 7

Needs for provision of information and communication in families

with adolescents in pediatric oncohematology

N.S. Nikolskaya, A.Ye. Khain

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: adolescence, provision of information, information needs, communication style, adaptation

Introduction. A modern comprehensive approach to healthcare delivery implies addressing the issues of psychosocial adaptation of children to the disease and treatment. A child’s

adequate age-appropriate perceptions of a disease have a protective function toward the risk of distress development and compliance violation during the treatment. The practice of

informing patients of the disease and treatment not only in pediatrics has been used rather recently within the traditions of national approach, so it raises an issue of communication

styles that would allow to inform children about the features of the disease and therapy. In the Russian Federation adolescents aged 15 and older have a right to give a voluntary,

informed consent. In spite of this, health care personnel often face parents’ unwillingness and resistance to inform adolescents about specifi c character of their disease and let them

take part in the discussion of the treatment process, its duration and the prospects. Communication approaches to providing information should take into account needs of the

adolescents themselves, their individual capacity to integrate the received information. The approaches should also be adjusted to sociocultural aspects and traditions. The study

of information needs of all the participants of treatment process appears to be of current interest and enables us to adjust existing foreign communication approaches to informing

adolescent patients and their parents better.

Aim. To investigate information needs of parents and adolescent patients in respect of disease and its treatment.

Materials and methods. Questionnaires were designed to identify the desired style of informing, information needs (form, scope, content, sources of information). Versions for

adolescents and their parents/legal guardians. Respondents: 1) parents of adolescents (n = 54, aged 32–63 years old; М = 43.7; SD = 9.1; 53 female and 1 – male); 2) adolescent

patients (n = 49, aged 14–18 years old; М = 16.4; SD = 2; 23 – female and 26 – male).

Results. The study has demonstrated the need of adolescents in building direct communication with a doctor rather than indirect communication through their parents. Adolescents

much prefer receiving information directly from a treating physician (p ≤ 0.05), and this fact is underestimated by their parents. Readiness of adolescents for getting information

about the severity of the disease and treatment is dependent on the quality of communication with a physician, the degree of the achieved trust (p ≤ 0.05). Vice versa, unwillingness

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to get information is signifi cantly related (p ≤ 0.05) to the main sources of information from Internet or directly from parents. Parents are more willing to let doctors inform adolescents

about their diagnosis rather than discuss with them the severity of treatment, prognosis or side eff ects of therapy (p ≤ 0.05), 37.5 % of them do not believe it possible at all to inform

their children. Adolescents concern themselves about the causes of the disease and the issues of direct participation in the treatment process, e.g. guidelines for hygiene, nutrition,

signifi cantly more than parents (p ≤ 0.05). Parents, however, are primarily concerned about future perspectives, the plan and prognosis of the therapy being administered (p ≤ 0.05).

Conclusion. The study demonstrates the importance of direct communication between adolescents and treating physicians while providing information for better adaptation rather

than indirect communication through parents. Adolescents declare their readiness to deal with illness and treatment with the help of healthcare personnel and parental backing,

whereas more than one third of parents do not consider it possible to inform their children.

A B S T R A C T N O . : P - 2 4 0

Level of psychological trauma in mothers of children with cancer

O. Chernenko, O. Ushakova, S. Oleshko, D. Bakun, Yu. Fedyukova, E. Morozova,

A. Gevorgyan, L. Zubarovskaya, B. Afanasyev

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation,First Pavlov State Medical University of St. Petersburg, Russia

Key words: psychological state of the mother, oncological disease of a children

Introduction. Serious illness of a child is a diffi cult challenge for parents. Parents who are permanently supporting the child during his treatment at a cancer clinic are faced with

strong emotional experiences. These experiences are associated not only with disease but also with the treatment process. Standard chemotherapy protocols are not always possible

to achieve stable remission of the disease. Autologous transplantation improves the results of treatment substantially, but high risk of developing life-threatening complications has

a negative impact on psycho-emotional state of the parents of the patients.

Aim. The goals of the study are as followed:

• to study of the psychological state of the mother of a seriously ill child;

• to analyse the impact of an oncological disease of a children the psychological state of the mother.

Materials and methods. During the research 32 mothers of children undergoing treatment at Raisa Gorbacheva Memorial Research Institutefor Pediatric Oncology, Hematology and

Transplantology and at the Cancer Research Institute by N.N. Petrov were studied. Diff erent kinds of methods were used, such as, but not limited to:

• clinicalpsychological interview, aimed at identifying the characteristics of the personal response of mothers to child’s illness;

• experimentalpsychological method: Impact of Event Scale – Revised (IES-R);

• the test of an assessment of self-actualization (POI);

• questionnaire for diagnostics of the mother’s reaction to an illness of the child.

Results. Research of level of traumatic stress (IES-R) allowed to determine three groups of mothers: with low (31.2 %), high (34.4 %) and extremely high (34.4 %) level of

a psychological trauma accordingly.

For mothers with extremely high levels of psychological trauma the sick child was signifi cantly more often the only one in the family and was undergoing therelapse treatment. Most

of the women in this group weren’t residents of Saint Petersburg. Analysis of the results of the assessment of self-actualisation (IES-R) has shown that mothers with high levels of

psychical trauma tend to express their feelings spontaneously and directly.

The higherthe degree of a traumatic stress, the more expressive is behaviour. This could often be shown not only in interaction with the child or relatives, but also with the other

people, fi rst of all, the medical personnel.

The study of the relationship of mother towards the child’s illness shows that mothers with high levels of trauma tend to feel powerless and unable to aff ect illness outcome. These

feelings increase proportionally to the duration of the disease, especially in case of a relapse.

Correlation analysis of the results showed that parents’ ability to cope with their psychological and emotional state is important for the successful and timely treatment of the child,

as in many respects determines the behavior of the sick child, and his relationship to treatment, and to the doctor.

Conclusion. Oncological diseases in children are traumatic experiences for most mothers. The way the traumatic experience is perceived is infl uenced not only by the mother’s

personality, but also by external factors, such as the stage of the disease, the act of relocating to a diff erent city with the purpose of getting access to the treatment, as well as the

overall spirit of the relationship inside the family. Psychological support has been providing not only patients but also their relatives during the passage of the child’s treatment in

our clinic.

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A B S T R A C T N O . : O P - 2 7 0

Evaluation of burnout syndrome in pediatric hematologists oncologists

T.V. Stepanova1, G.V. Trubnikova2, I.Yu. Balalaeva2, N.B. Yudina2 1Voronezh State Medical University named after N.N. Burdenko, Russia;

2Voronezh Regional Children’s Clinical Hospital № 1, Russia

Key words: emotional burnout syndrome, pediatric oncologists, primary care physicians

Introduction. According to the International Classifi cation of Diseases (ICD-X): Burnout is stress associated with diffi culty maintaining a normal lifestyle (category Z73). Defi nitions

of burnout syndrome are: Burnout is a prolonged response to chronic emotional and interpersonal stressors on the job, and is defi ned by the three dimensions of exhaustion, cynicism,

and ineffi cacy (Maslach,1976). Emotional burnout – is a psychological defense mechanism in the form of full or partial exclusion of emotion in response to the traumatic eff ects of

stressors (Boyko,1999). Prevalence of burnout among physicians is as follows: 27 % of residents demonstrates signs of burnout; primary care physicians has burnout in 46.3 %. Among

cancer clinicians in Switzerland, 33 % expressed signs of emotional exhaustion. In Russia Pediatric Oncologists and Hematologist might be at higher risk of burnout.

Aim. To evaluate the level of burnout in Pediatric Hematologists Oncologists and to compare the level of burnout Pediatric Hematologists Oncologists with that in primary care

physicians and Pediatric residents.

Materials and methods. Cross-sectional pilot study was performed in Voronezh N.N. Burdenko University Hospital in January 2010 – January 2013. The were three study

groups under investigation: 1. Pediatric Hematologists Oncologists (n = 13); 2. Primary Care Pediatricians (n = 17); 3. Pediatric Residents (n = 13). We use the Questionnaire

“Burnout syndrome” V.V. Boyko. The basis of the questionnaire is the assumption that the burnout phase includes the “tension”,“resistance” and the “exhaustion”. Score interpretation

for phases: from 36 points to 60. Each of the phases includes a range of symptoms 1 – tension: Stressful circumstances; Dissatisfaction with oneself; “Drive into the cage”; Anxiety

and depression. 2 - Resistance: Inadequate response; Emotional disorientation; Emotional economy; Professional reduction. 3 - Exhaustion: Emotional defi cit; Emotional detachment;

Personal detachment; Psychosomatic disorders. The severity of each symptom was assessed on a scale 0 to 30 points.

Results. The distribution of the total score in study groups was: in Pediatric Hematologist Oncologists –143 points; in Primary care Pediatricians – 130; in Pediatric Residents – 83.

Tension phase score in study groups was not diff erent. The Resistance phase score was higher in Primary care Pediatricians. According to phase formation in Pediatric Hematologist

Oncologists number of formed phases were: one – in 15.3 %; two – in 7.6 % and all – in 7.6%. In Primary care Pediatricians only one phase (Resistance) is formed in 47 %. In Pediatric

Residents phases of burnout are not formed.

According to summary score and phase formation Burnout syndrome was diagnosed in 38.5 % of Pediatric Hematologists and in 11.7 % of Primary care physicians. Spearman rank

correlation coeffi cient demonstrated expression of the tension (score) by profession r = 0.5, P = 0.000; expression of exhaustion (score) by profession r = 0.6, P = 0.004. Expression

of the total score by profession was r = 0.8; P = 0.000.

Conclusion. Burnout syndrome was defi ned in 38.5 % of Pediatric Hematologists and in 11.7 % of Primary care physicians. Dominant symptoms of burnout for each phase were

identifi ed and the severity was assessed.

A B S T R A C T N O . : O - 2 8 0

Reproductive behavior of families with children-cancer survivors

M.A. Guseva1, G.Ya. Tseitlin1, A.I. Antonov2

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2M.V. Lomonosov Moscow State University, Russia

Key words: reproductive behavior, paediatric oncology, parents, family

Introduction. Childhood cancer and its treatment infl uence both individuals within the family and the family as a whole, in particular its reproductive strategy. This aspect of family

functioning is poorly understood.

Aim. The purpose is to study reproductive behavior of families with children-cancer survivors.

Materials and methods. Data from the families were collected through individual interviews and questionnaires of 1085 mothers from 78 regions of the Russian Federation;

mothers aged 25–49 (average 36.6), with a cancer child aged 5–17, in remission from 1–12 years (average 4.2). The results were compared with the data of the Russian state statistics

agency’s demographic research of the same period – 1118 female respondents of the same age range (the control group).

Results. The number of born children in our group considerably diff ered from the control: 1 child – 39.7 % vs 58.3 %; 2 children – 48.7 % vs 27.8 %; 3 – 9.6 % vs 3.8 %;

4 and more – 2.1 % vs 0.7 % respectively. The indices of reproductive attitudes in our group were higher than in control: average desired number of children – 2.59 vs 2.28; average

expected number of children – 2.05 vs 1.72 respectively. Family values – family moorings and relationship, ties, household, possession of many children in our group took higher rank

places than in control. Childhood cancer impacts on parents’ relationship: 25.3 % – improved; 10.7 % – deteriorated; 9 % – divorced; 55 % – no change. Chronical emotional stress

considerably impacts on reproductive health: 12.5 % had reproductive function’s disorders.

Conclusion. It is suggested that childhood cancer changes the meanings and life priorities towards a pro-family orientation, considerably increasing the need in children. The families

need psychological, social and medical support to overcome stress, improve their health and quality of life.

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A B S T R A C T N O . : O - 2 8 7

Psychological service development of PBHF of SO ODCH 1 Children’s Oncology

and Hematology Centre

T.A. Ziskelevich, I.S. Savran, S.V. Logvinenko, А.О. Kuznetsov

Regional Children’s Clinical Hospital № 1, Yekaterinburg, Russia

Key words: psychological service, psychological aid, oncopsychology

Introduction. Initially psychological service (PS) creation project was planned not only in a narrow sense of aiding to oncology patients, but also as a structure, aimed at outreach

and educative activities in the fi eld of oncological problematic, both in the framework of professional environment and in the society.

Aim. Formation of aims was carried out in several directions: a psychological aid to children and parents, a rehabilitation course, integration issues of PS in medical sphere of

healthcare centre, preparation and education of professionals.

Materials and methods. For 5 years of PS work there were defi ned groups of issues that were solved during work: issues in the fi eld of dyadic mother-child intercourse, oncology

subject adaptation at the social level, burnout, prevention of rehabilitation, acquisition of diff erent social institutions for the coverage of oncology issue of the society, professional

improvement. One of the main principles of PS work is individual and collective responsibility to society. A key aspect in service organization and functioning is a well-structured

system formation which includes general principles of process organization, statistics, structuring of PS-addressed-requests, a psychological instrumentation set that is authentic

to professionals, well-defi ned work strategy and concept, distinct requirements set to professionals. Such work process structure allows to support a predictable work space with

well-defi ned lines, which is the foundation of stable and long PS functioning at high professional level. Along with this clear frames allow to detect problematic zones in PS work.

Results. Defi nition of PS work perspectives/opportunities is very important, because in our opinion absence of clear perspectives of PS development during work in this fi eld leads

to fast stagnation and burnout of the system itself in very short terms. This may be one of the obstacles in PS creation and functioning in the oncology fi eld. Shortness and burnout

prevent scientifi c site creation and experience exchange, i.e. the processes that are the basis of scientifi c oncopsychology school creation in Russia.

Conclusion. Nowadays we managed to integrate PC into Children’s Oncology and Hematology Centre, to actualize needs of patients, their relatives and medical staff in psychological

aid in oncological in-patient facility, to lay a rehabilitation basis for the period of inpatient stay till the moment of release.

A B S T R A C T N O . : O P - 3 1 6

The stigma of cancer in developing countries

Atish Narayanrao Bakane, Ramya S Uppuluri, Divya S Subburaj, Sreejith R Kurup, Revathi R Raj

Apollo Speciality Hospital, India

Key words: developing countries, diagnosis of childhood cancer, retrospective oral questionnaire, psychosocial aspects of cancer

Introduction. The diagnosis of cancer is seldom disclosed to children or discussed with them in developing countries after completion of chemotherapy. We present here data on the

extent and patterns of social stigma associated with the diagnosis of childhood cancer in middle income families in India after completion of therapy.

Aim. Data on the extent and patterns of social stigma associated with the diagnosis of childhood cancer in middle income families in India after completion of therapy.

Materials and methods. This a retrospective oral questionnaire done on parents of children treated in a tertiary cancer centre in India over a ten year period between 2002 and 2012.

The children who were less than 7 years old during therapy and were in remission over 3 years were chosen as our study population. The parents were questioned on 3 parameters –

discussion of the treatment of cancer with the child, attitude towards follow up care and fear regarding long term side eff ects of the treatment.

Results. A total of 59 parents were interviewed for the study. The diagnosis was Acute Lymphoblastic leukaemia in 37 children, Hodgkins lymphoma in 5 and non Hodgkins lymphoma

in 10 children. Only two parents had discussed the diagnosis of cancer with their child (3.4 %). Follow up was not off ered for fear of sensitive questions from the children in 83 % of

children and only 10 children have been coming for regular annual follow up since discharge. All parents – 100 % expressed fears about delayed side eff ects from the chemotherapy

especially on pubertal growth and fertility but did not wish to discuss this issue with the child. Separate appointments were made to perform a chart review in 12 % patients by the

parents to address this issue of late side eff ects.

Conclusion. The social stigma of cancer chemotherapy in our society has resulted in a huge loss of data on the psychosocial aspects of cancer and its impact on the family. More COPE

programs – creating opportunities for parent empowerment are required to overcome these barriers and help support these families long term.

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A B S T R A C T N O . : P - 3 3 5

Psychiatric issue of “medical stress” of parents in pediatric oncology/ hematology

S.N. Masikhina

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: adjustment disorder, stress-ralated disorders, anxiety, depression, parents, adherence, pediatric oncology

Introduction. A treatment success in pediatric oncology depends largely on the parents that are “intermediaries” between the child and the system of health care. Untimely

treatment, violations of the drug therapy, treatment failures and defects in care are essential factors of poor prognosis of cancer. Among the factors of low adherence to treatment are

the individual characteristics and psychopathological symptoms.

Aim. The clinical study of the role of mental disorders in the structure of “medical stress” in parents of children with cancer and impact on treatment adherence.

Materials and methods. There was conducted a screening study of 428 parents of children with cancer (solid tumors, leukemia and lymphomas) who have agreed to participate

in the study. The study was conducted by using Hospital Anxiety and “Depression Scale” (HADS), Morisky Medication Adherence Scale (MMAS) and additional items to assess the

quality of care. The mean age of parents was 34 ± 7.8, length of hospital stay from 1 to 6 months. 82 parents (69 mothers, 13 fathers) were selected among the total sample that

scored more than 11 points on HADS (HADS-A and HADS-D). There were excluded parents of children in terminal conditions, and parents with pre-existents conditions as chronic

alcoholism, mental retardation or organic brain disease. The analysis focused on premorbid characteristics, clinical patterns and dynamics of “medical stress”-related disorders. The

obtained data were correlated with the diagnostic criteria of ICD-10. Comparison of mean values and the proportion of paired values was conducted by using Student’s criterion (level

of signifi cance < 0.01).

Results. According to clinical analysis of “medical stress”-related disorders in parents were identifi ed: adjustment disorders (AD) – 48.7 %; decompensation of personality disorders

(DPD) – 34.1%; mood depressive disorders (MDD)- recurrent depression, dysthymia, moderate depressive episode –10.9 %; exacerbation of schizophrenia spectrum disorders

associated with stress (SSD) –6 %. The main clinical and psychosocial characteristics of the groups shows the diff erences in terms of adherence to treatment. Comparative analysis of

the MDD group showed: parents less likely to comply with hours of medication and (M = 2, SD = 0.22 and M = 48, SD = 0.96, t = 8.3 psychotic symptoms, cognitive disorganization

signifi cantly reduced rates of adherence to therapy.

Conclusion. Mental disorders in parents has a negative impact on the treatment of cancer in children. The impairment of therapeutic cooperation were the most signifi cant in the

groups DPD and SSD. In clinical practice, it is necessary to identify in advance psychiatric disorders in parents to improve the quality of medical care. Medical staff need to adhere to

certain rules of psychosocial interaction with parents with mental disorders.

A B S T R A C T N O . : P P - 3 9 7

Subculture groups of children in medical institution in aspect of interaction with parents

communities and public organizations

I. Ageeva-Podobed, G. Kireeva

Chelyabinsk Regional Children’s Hospital, Russia

Key words: subculture, children, therapy

Introduction. Forming of subculture inside of children’s groups in medical institution connected with children’s adaptation and rehabilitation that have serious illness and needed

in restoration to health, education and assigning educational values.

Aim. To confi rm the eff ect of formation healthy lifestyle in subculture groups of children in medical institution.

Materials and methods. Researches of subculture children’s groups were made in the unit of pediatric oncology and hematology of Pediatric Hospital of Chelyabinsk Oblast.

Interconnections between public organizations, parent’s communities and subculture groups of children were studied.

On the basis of the studied material plan of the creative associations in offi ce of play therapy was built. Research was made in conjunction with specialist from unit of pediatric

oncology and hematology of the Pediatric Hospital of Chelyabinsk Oblast. Most active among them were educational psychologists, educators, volunteers.

Results. The culture of relations in the hospital is sophisticated and dynamic phenomenon which depends on level of forming culture of communication relations children’s group but

also in medical institution, their focus on socio-cultural values.

Culture of relations in isolated social groups is a subculture. During hospitalization in the unit of oncology and hematology besides medical treatment patient require his or her

everyday life. During medical treatment it is important to mobilize internal psychological, physical, intellectual reserves of the children and to form the orientation of the personality

in socio-cultural values – healthy way of living.

Conclusion. Subculture as a kind of socialization is a social phenomenon which impacts on recover. It connected with the occurrence of the personality in educational space, with

interaction with his or her values and assignment, i.e. including them into the structure of personality. This requires a cultural approach as methodological principle of socialization

of children during medical treatment in hospital.

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A B S T R A C T N O . : O - 1 4 0

The usage of central venous access devices to improve the quality care in children,

treated with intensive chemotherapy

A.V. Petrichenko, I. Logachova, K.F. Savlaev, A.V. Zabrodnaya

Applied Research Center for Specialized Medical Care of Children named after V.F. Voyno-Yasenetsky, Russia

Key words: central venous access devices, pediatric oncology

Introduction. The requirements for the qualifi cations of the nursing staff of modern pediatric oncological department are extremely high. It is currently impossible to achieve best

results of treatment of children with solid tumors, without the usage of modern treatment protocols. The treatment plan for patients with solid tumors included innovative methods

of anticancer therapy, supportive treatment and high quality of nursing care

Aim. Optimization of medical care for children treated from solid tumors.

Materials and methods. The data were collected from patients who received the treatment for solid tumors at pediatric oncological department during 2013–2015 years.

A central venous access device plays an important role in the management of cancer patients. They serve not only for safe administration of chemotherapy, but also for prolonged

administration of fl uids, blood and blood products, antibiotics, parenteral nutrition, and frequent blood sampling. Diff erent types of central venous access devices are associated with

diff erent patterns of complications. There are diff erent types of venous access devices, but totally implantable venous access ports (TIVAP) are now used most commonly because

of their safety, cosmetics, low infection rates, ease of implantation, and usage. A proper TIVAP not only provides secure vascular access for all patients’ therapeutic needs, but can

also reduce the frequency of venipuncture for the purposes of native vessel protection. In this study, 75 patients at the age from 9 months till 18 years received port implantations.

Results. However, the procedure and its subsequent maintenance are not free of side eff ects –14 (27 %) TIVAP were removed prematurely due to complications. We had observed

complications associated with TIVAP, such as thrombosis and blockage at 11 patients (21 % cases), embolization at 2 patients (4 % cases), and catheter fracture at 1 patients

(2 % cases). We do not observed important complications, such as infections, pneumothorax, hematoma.

Conclusion. Intravenous ports are very important for children with solid tumors. The complication rate has decreased not only with improved techniques and material, but also high

quality of nursing care. With increasing experience and knowledge about TIVAP care, we hope the associated complications will decrease, resulting in improved patient safety and

compliance with the device.

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How to organize the best nurses care of venous catheter

D.Sh. Bikkulova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: hand skills, training dummy, commitment to the protocol

Introduction. Federal Research Centre of Pediatric Hematology, Oncology and Immunology began accept of patients in a new building in January, 2012. The medical staff had

experience, skills and knowledge in diff erent areas of medicine, not only hematology and oncology. Hematology and oncology patients have certain specifi cs: need CVC, receive

chemotherapy and steroid therapy. As a result patients have immunosuppression, metabolic disorders, fragile skin. These patients have a high risk of developing the catheter-related

infections of bloodstream. Nurses has to have additional knowledge in the area of hematology/oncology, good hand skills to support aseptic conditions.

Aim. It was important to defi ne uniform maintenance and care of central venous catheters (CVC).

Materials and methods. The solution of CVC problems is possible if the hospital have Internal protocols of venous access, Clinical recommendations of vascular access, full course of

lectures about catheters, a training class for nurses. CVC checklist is also needed.

Training component: nurses study to master hand skills, to remember the protocol, to know complications and prevent them, maintenance and care of CVC. Newly employed staff is

allowed to start work after mastering hand skills on the silicon dummy.

Task force were created in for preparation and adjusting protocols for our local needs. Task force consists of reputable employees: vice-chief of the hospital, anesthesiologists,

hematologists, epidemiologists from the Department of Infection Control, nurses. Discussions about measures on prevention the central line-associated bloodstream infections

(CLABSI) was especially diffi cult and long. The market off ers a big set of dressing for catheters fi xation. It is important to defi ne what dressing to apply on the catheter area. It is

necessary that all members of the task force would agree with off ered solution. Some members of the task force were not satisfi ed with scientifi c publications only. Several points of

the CVC protocol were accepted after additional tests or clinical trials conducted in our Hospital.

Results. We have protocols : No. 1 – the intraosseous vascular access, No. 2 – the Short peripheral catheter, No. 3 – Short-term CVC, No. 4 Long-term tunnelled CVC, No. 5 Long-

term implanted CVC, No 6 PICC-line. Adoption of protocols in medical center is long and laborious process. Staff has various experience in the medical area and habits in their clinical

practice. Agreement on certain opinion among the staff is easier to achieve through increasing level of knowledge, additional trial and search of compromises.

Conclusion. It is very important to have training class with tailor’s dummy for to master hand skills, CVC protocols, commitment to the protocol for the best nurses care and

maintenance CVC.

A B S T R A C T N O . : O - 1 8 9

The usage of innovative methods of sanitary disposal of the surgical unit

A.V. Petrichenko, I. Ozhogina, K.F. Savlaev, A.V. Zabrodnaya

Applied Research Center for Specialized Medical Care of Children named after V.F. Voyno-Yasenetsky, Russia

Key words: surgical unit, cleanings, disinfections

Introduction. The timeliness of the problem is based on the necessity of reduction of the use of cleaning and disinfecting agents, water consumption, and decrease of pressure on

medical stuff by using ergonomical inventory and scientifi cally based organization of cleaning process with the application of innovative technologies.

Aim. Prevention of post-surgery complications and hospital infection.

Materials and methods. From 2013 till the December of 2015 the system of harmless cleaning is applied in the surgical unit, it is used for all the cleanings and disinfections.

The inventory is made of polymeric materials, it can stand the use of disinfecting agents and autoclaving and has color coding. Previously dampen expendable cotton materials are

used on the strictly defi ned area, it keeps the constant concentration of disinfection solution what leads to the prevention of cross contamination and also to the prevention of splash

of contaminated liquids. As cleaning is over the inventory needs sanitary disposal for its future use. The system can be used with any means which are appropriate for the cleaning

of operation unit.

Results. 153 deep cleanings and 2 964 ordinary cleanings are made. The use of the system allowed to reduce the stuff by 67 % and to reduce the time of cleaning for 2.6 times. Duly

made washouts and inoculations showed negative result in all cases. The quantity of sanitary indicator microorganisms using all the methods of cleaning amounted to 0 % whereas

the norm before the start of the work is 200 CFU/cm and the norm during the work is 500 CFU/cm. Performed 497 operations in 460 children with solid tumors, from 2013 till 2015

years. Early postoperative complications caused by an infectious process were observed in 7 (1.5 %) cases.

Conclusion. The innovative system of harmless cleaning is aimed to the prevention of cross contamination, the spread of hospital infection and infectious post-surgery complications.

It allows to optimize the stuff and the time of cleaning, reduce the terms of preparation for cleaning and it can signifi cantly improve the epidemiological conditions of the operation unit.

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A B S T R A C T N O . : O - 0 7 6

Perfection of the organizational-methodological approaches to care

for children with cancer in the Russian Federation

M. Rykov, V. Polyakov

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia

Key words: pediatric oncology, organization of medical care, epidemiology of malignant tumors, quality management of medical care

Introduction. Despite the fact that malignant tumors are the second most common cause of death for children in pediatric oncology signifi cant progress. However, the large size of

the Russian Federation, diff erent size and density of the child population in the regions of the country require careful planning organization of cancer care for children and the rational

use of available resources.

Aim. Perfection of the organizational-methodological approaches to care for children with cancer in Russia in order to improve the quality and eff ectiveness of treatment.

Materials and methods. In a non-randomized study included reports of regional ministries and departments of health of 83 subjects of the Russian Federation for 2014.

Results. The highest incidence of malignant tumors (per 100 thousand aged 0–17 years) registered in the Lipetsk region – 21.7, the lowest – in the Republic of Tyva – 5.5.

The number of patients with newly diagnosed in 2014 was the highest in the Central and Volga federal districts – 775 and 653, respectively. The smallest – in the Far Eastern

Federal District – 138. The number of primary patients referred to the federal clinic, was the largest in the North Caucasus Federal District (North Caucasus Federal District) – 80 %,

the lowest – in the South – 32.5 %, the largest number of doctors who do not have primary specialization in “Children’s oncology”, registered in the North Caucasus Federal District and

the Volga Federal District – 50 %, the lowest – Urals – 14.2 %. Total in Russia operates 51 children’s oncology department, while the number of beds in which we treat these patients,

including beds in non-core branches is 2021. The number of doctors who treat children with cancer, is 390, of which 252 (64.6 %) did not have a certifi cate of pediatric oncology.

In 2014, 3378 children were registered with the External testing, of which 1705 (50.5 %) were directed for treatment in federal clinics. Features of high-dose chemotherapy, there are

36 (42 %) of the 85 subjects of the Russian Federation, radiation therapy – in 62 (73 %) with the restriction of the lower limit of age (6–15 years), high-tech and organ operations –

only the federal government.

Conclusion. Requires further improving the existing vertical provision of high-tech medical care for children with cancer, the creation of regional cancer registers, certifi cation of

specialists and improving the quality control of medical care through the implementation of internal and external audit.

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Spontaneous tumor rupture in children: a rare event with fatal outcome

Nabila Bouterfas1, Ayda Mohand Oussaid1, Zoulikha Zeroual1, Yasmine Bouskia1, Amghide Khati1,Arslane Slimane Taleb1, Nassima Merazi2, Mohamed Elmokhtar Khiari1, Houria Boukhelal1, Nafi ssa Keltoum Benhalla1

1CHU Beni Messous; 2Hospital Mustapha Pacha, Alger, Algeria

Key words: solid tumors, neuroblastoma, nephroblastoma, acute myeloblastic leukemia

Introduction. Tumor rupture is a dramatic event described in children presenting mostly with solid tumors.

Aim. To report on clinical characteristics of patients presenting with this complication, to analyze treatment features, and to evaluate outcome.

Materials and methods. We retrospectively analyzed the data of 12 children diagnosed at the Pediatric Oncology Unit of CHU de Beni Messous from 1st January 2005 to 31th

December 2015 as having a haemorrhage or/ and a tumor rupture. All patients were evaluated by a physical examination, with a hematological exploration, and were documented

with imaging including abdominal US or CT scan.

Results. Tumor rupture was essentially seen in patients presenting with neuroblastoma (9 cases). There were 2 cases of nephroblastoma and only one case of acute myeloblastic

leukemia that developed a splenic rupture.

Severe pallor and acute abdominal pain were the principal alarming symptoms. Imaging fi ndings demonstrated an intraperitoneal haemorrhage in all patients.

Approximately 40 % of patients exhibited this manifestation at initial diagnosis, in the rest; the diagnosis was made during the evolution.

20 % of patients underwent surgery, consisting on nephrectomy principally in patients presenting with Wilms’ tumor, while the others had a conservative management because of

general conditions instability.

Survival rates were extremely poor, only that patient with splenic rupture is still alive with a follow up time of 3 years.

Conclusion. Tumor rupture is a real complication aff ecting the behavior outcome of patients with malignancies, since it’s particularly associated with increased mortality. This study

also showed that rupture is more frequent in children with neuroblastoma.

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Childhood solid tumors incidence in the Kyrgyzstan

E. Makimbetov

National Cancer Center, Bishkek, Kyrgyzstan

Key words: solid tumors, incidence, crude rate, age-standartized rate

Introduction. There were a geographical, ethnic and age specifi c variability of childhood cancer incidence, include solid tumors.

Aim. To study age-specifi c, regional and ethnic childhood solid tumors incidence in the Kyrgyzstan.

Materials and methods. Calculated the incidence of childhood solid tumors between 2000 and 2014. There were collected a clinical, histological, cytological dates, and deaths

certifi cates. Estimated population relative risk (RR) for the solid tumors in children at the urban and rural areas, also ethnic groups. Counted crude, age-standardized rates

(ASR) per 1 000 000.

Results. There were registered 3225 with a new diagnoses of cancer in children (0–14 years).

Total annual childhood cancer incidence was 83.6 per 1 million. The most frequent diagnostic groups were leukaemia’s (33.6 %, ASR 24.7) and lymphomas (15.4 %, ASR 10.3).

Solid tumours registered in 1653 children (48.9 %). Central nervous system tumours were on the 1st place with ASR 8.1 (284 cases, 150 in male and 134 in female). Wilms tumour was

on the 2nd place with ASR 5.4 (205 cases). Soft tissue sarcomas (ASR 5.1) and bone tumours (ASR 4.7), sympathetic nervous system and neuroblasomas (ASR 4.6) and retinoblastoma

(ASR 3.8) were the main solid tumours among childhood cancer. Histological verifi cation in solid tumours was 93 %.

Solid tumors incidence was signifi cantly higher in the Bishkek and Chui regions (ASR 50.0–60.0). Average incidence rate was registered at the Naryn, Isyk-kul (ASR 30.0–40.0) areas.

Low incidence rate was registered at the Osh, Galalabat, Talas and Batken.

Conclusion. Childhood solid cancer incidence in the Kyrgyzstan is very low and similar to those reported from some world developing countries.

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Childhood cancer morbidity in the Republic of Mordovia

E.O. Zauralov1, M.G. Galkina1, I.А. Ruchina1, V.S. Vereshchagina2

1Children’s Republican Clinical Hospital, Saransk, the Republic of Mordovia;2Ogarev Mordovia State University, Saransk, the Republic of Mordovia

Key words: children, cancer morbidity, death rate

Introduction. Among the problems of modern pediatrics, malignant neoplasms (MN) occupy one of the leading positions. Over the past decade in Russia, the number of children

being under observation due to malignant neoplasms (MN) of all locations has increased by 20 %.

In the Republic of Mordovia (RM) childhood cancer morbidity for a 14-year period (2000–2013) has also increased by more than 12 %. In this regard, the investigation of regional

specifi cities of the morbidity and mortality of MN in children in the Republic of Mordovia acquires particular signifi cance.

Aim. To investigate cancer morbidity and mortality in children aged 0–17 years old living in the Republic of Mordovia for the period 2000–2013.

Materials and methods. The analysis of malignant neoplasm incidence over time in children from the RM, from 2000 until 2013, was carried out. We used the following sources of

information: primary notifi cation about newly diagnosed oncological disease, anamneses and medical records of children who underwent treatment in the oncological department of

Children’s Republican Clinical Hospital of Saransk, as well as the data from the Republican Oncologic Dispensary and the Organizational and Methodological Department of Children’s

Republican Clinical Hospital.

When investigating cancer morbidity, the International Classifi cation of Diseases, 10th Edition, was used. The Student’s t-test was applied for determination of statistical signifi cance

of diff erences between compared indices. For the statistical analysis Statistica 10.0 was used.

Results. Over the period 2000–2013, 378 children with oncologic diseases were registered in the Republic of Mordovia (RM). Despite the signifi cant decrease in the child population

during this period, cancer morbidity was consistently increasing from 10.1 per 100 000 of child population in 2000 to 16.0 per 100 000 of child population in 2013. The average disease

incidence in children from the RM over 14 years amounted to 17.58 per 100 000 of child population. The mean annual increment of disease incidence amounted to 4.8 %, total growth

was 79 %. The neoplasms of hemopoietic and lymphatic tissues take the 1st position of overall incidence of cancer (7.73), and central nervous system tumours take the 2nd position

(2.85). Mortality rate due to oncologic diseases in children from the RM also has increased over a period of 14 years. In the structure of mortality cases of children with leukemia and

lymphomas prevailed (3.29).

Conclusion. During the investigation two age groups of children were determined: 0–4 years old and 15–17 years old (adolescents) where the disease incidence was signifi cantly

higher in comparison with other age groups (25.6 and 18.34 per 100 000 respectively). In comparison with neighboring regions, the RM has the highest cancer morbidity rate

(17.58 per 100 000) and one of the lowest mortality rates of oncological diseases in children (4.1 per 100 000).

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Molecular epidemiology of persister populations of opportunistic bacteria isolated from

children with hematological malignancies

A.M. Gaponov1, A.V. Tutelyan1, I.A. Alexandrov1, A.G. Chumachenko2, V.M. Pisarev2

1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia; 2V.A. Negovsky Scientifi c Research Institute of General Reanimatology, Moscow, Russia

Key words: molecular epidemiology, persister populations, opportunistic bacteria isolated

Introduction. Persisters (Ps) are dormant forms of bacteria capable to survive deadly antibiotic treatment, nutrient defi ciencies and oxidation stress generated by infl ammatory

microenvironment without acquisition of drug resistance-specifi c mutations. Most recently, Ps has become an attractive area of studies due to their evident impact on health care

especially in immunocompromised patients. It is expected that the Ps do determine the increasing number of chronic infectious diseases such as tuberculosis, infective endocarditis,

Haemophilus infl uenzae infection, catheter-associated pneumonia, and presumably health care associated infections. Surviving bacterial populations following the action of lethal

doses of potent antibiotic is provided by the presence of a small (0.01–1 %) subpopulation of antibiotic-tolerant (AT) Ps, which resistance to antibiotics is reversible, unlike in classic

antibiotic resistant bacterial cells. Rapid entry in a dormancy helps Ps to survive unfavorable microenvironment and “resuscitate” later, with capabilities to grow and spread.

Aim. Elucidation of the mechanisms mediating the formation of PS within opportunistic bacteria that can cause sepsis or hardly treatable chronic infection

Materials and methods. In this study, the Ps frequencies within the clinical isolates of opportunistic pathogens E. coli and P. aeruginosa isolated from children with hematologic

malignancies (acute lymphocytic leukemia – ALL and chronic lymphocytic leukemia – CLL) were estimated and molecular characterization of genes presumably involved in Ps

generation was determined. Total of 89 strains were isolated from specimens of children with ALL and CLL (ages from 1 year to 17 years) admitted to clinical departments of Dmitry

Rogachev’ Federal Scientifi c Clinical Research Center of Pediatric Hematology in 2012–2015: E. coli – 47 strains, P. aeruginosa – 42 strains. Additionally, 12 strains of E. coli and 8 strains

of P. aeruginosa were isolated from parents of patients.

Results. About a third of the isolated strains were characterized by a high content of Ps determined by a rapid recovery following high-dose antibiotic attack in vitro. The insensitivity

of Ps fractions to ciprofl oxacin was 100–1000 times greater than one in a reference level strain. Gene sequencing of Ps DNAs demonstrated the skewed profi le of promoter regions from

diff erent operons including point mutations and deletions within relA, spoT1, spoT, dnaK, dnaG, rpoS promoters compared to non-Ps clinical isolates of E. coli and P. aeruginosa. We

believe that profi ling the allelic variants and expression of genes that presumably contribute to Ps generation and/or survival will help to clarify the risk factors for persisting infection

and determine novel molecular targets to defeat Ps and discontinue the chronic or repeated infection in immunocompomized children.

Conclusion. This study was supported by a grants 13-04-40230-Н (COMFI) and № 13-04-40229 from Russian Foundation for Basic Research.

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Epidemiology of ovarian neoplasms in girls of diff erent age groups

in the Republic of Bashkortostan

S.Yu. Muslimova1, V.B. Makhonin2, R.R. Bayramgulov2, G.Yu. Battalova1

1Bashkirian State Medical University, Ufa, Republic of Bashkortostan2Republican Children’s Clinical Hospital, Ufa, Republic of Bashkortostan;

Key words: epidemiology, neoplasms of the ovaries, girls

Introduction. Modern data on the epidemiology and clinic of tumors and Ovarian Tumor formations in children are contradictory. In the few works dedicated to this topic, is generally

considered the results of the analysis in a small, statistically insignifi cant, disparate age groups of patients who received in-patient treatment in clinics and offi ces of various profi les.

As a result, rates of tumors and tumor formations of ovaries in girls range widely, from 0.5 up to 13.3 %.

Aim. To study the epidemiology characteristics of ovary neoplasms in girls of diff erent age groups.

Materials and methods. We used a retrospective analysis of the case histories of children from 0 to 17 years, who were treated in pediatric gynecology and pediatric surgery

departments of the emergency hospital in Ufa and in the Republican children’s clinical hospital during the period from 1992 to 2012. Patients were divided into groups by age periods

in accordance with the main stages of formation of the reproductive system of women, according to the classifi cation approved by WHO in 1965 and revised by E.V. Uvarova in 2009.

Results. During 20 years-period, the number of girls with tumors and tumor-like formations of the ovaries, who entered the Republican hospitals, had increased in 2.5 times. The

annual number of newborns hospitalized in the Department of newborns surgery increased from 1 to 16 cases, and the proportion of girls in I and II phases of puberty among those,

who were hospitalized in the Department of pediatric gynecology had increased from 4.1 to 10.3 %. The number of girls in the period of childhood with the same pathology had not

changed. The sickness rate of superfi cial epithelial-stromal, germ-cell tumors and tumor-like neoplasms of the ovaries was signifi cantly higher in girls living in large industrial center

than in children from small towns and rural areas. Among the examined patients there were equally often met girls with tumor-like formations of ovaries 49.33 % (n = 512) and true

tumors 48.67 % (n = 523). At 1.99 % (n = 21) of patients because of necrosis of an ovarian tissue caused by the torsion, the histological structure was not identifi ed. In 89.4 % of cases

(n = 456) tumors in girls were benign, borderline tumors were detected in 3.12 % of (n = 16) and malignant – at 8.18 % (n = 42). The frequency of ovarian neoplasms in girls was

proportional to age – the older the age, the greater the number of patients. The structure of tumors and tumor-like formations of the ovaries also depended on the age of the girls,

and signifi cantly changed in the year of menarche. If newborn girls and puberty in 92 % and 59 % of cases had the surface epithelial-stromal tumors, then in the period of childhood

and pre-pubertal period dominated germ-cell tumors – in 60 and 63 % accordingly. The malignant ovarian tumors prevailed in girls and pre-pubertal children of age periods – in 11

and 14 % of cases accordingly.

Conclusion. Thus, there were marked the incidence rate of ovarian neoplasms in infants and girls in puberty, higher rates of children living in large industrial city, the relationship

between the prevalence of tumors and their structure and age of patients.

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The incidence of the most common malignant tumors of childhood

in the Chelyabinsk region for the 8-year period

S.G. Kovalenko, I.I. Spichak, E.V. Basharova, K.V. Volkova, D.I. Toporkova

Chelyabinsk Regional Children’s Hospital, Russia

Key words: leukemia, CNS, incidence, Chelyabinsk

Introduction. The child population of Chelyabinsk region resides mainly in major industrial cities and is, therefore, exposed to a variety of anthropogenic and natural adverse factors.

Active development of regional pediatric cancer care calls for extra attention in epidemiological indicators monitoring as it is necessary for adequate care planning and therapy results

improvement.

Aim. The aim of the study was to determine the dynamic in the incidence of the most common types of childhood cancer in the Chelyabinsk region.

Materials and methods. The regional registry of children cancer was established on the base of Chelyabinsk regional children hospital, since 1996 prospective data collection was

initiated and starting from 2008 all cases of childhood cancer (patients of 18 years or less) were prospectively registered. For grouping purpose we used International Classifi cation of

Childhood Cancer (3th revision). Population data were obtained from offi cial website of the state statistical offi ce.

Results. In the analyzed period we have registered 185 patients with acute lymphoblastic leukemia (ALL), 177 aged 0–14 years, and 105 patients with tumors of central nervous

system (CNS), 88 aged 0–14 years. The age-standartized rate (world standard) for ALL ranged between 3.13 and 5.24 per 100000, for CNS-tumors between 1.30 and 2.97 per 100000.

For last 2 years (2014–2015) we noticed increasing of incidence of ALL (ASR 5.24 and 4.9 per 100 000 respectively, P = 0.015). In age groups, we noticed the predominantly increasing

of incidence in group 1–4 years (P = 0.007). No any signifi cant deviation in incidence of CNS tumors was registered for the study period.

Conclusion. Our preliminary results of the study requires further monitoring of the epidemiological situation and the careful study of the entire complex of anthropogenic, geological

and cosmogenic factors in Chelyabinsk region.

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Survey on pediatric cancer patients in Kanto-Koshinetsu area after the election

of Childhood Cancer Core Hospitals in Japan

Kimikazu Matsumoto1, Yuki Yuza1, Hiroaki Goto2, Katsuyoshi Ko3, Daisuke Tomizawa4, Takashi Kaneko1, Ryoji Hanada3 1Tokyo Metropolitan Children’s Medical Center; 2Kanagawa Children’s Medical Center;

3Saitama Children’s Medical Center; 4National Center for Child Health and Development

Key words: medical system, patient survey

Introduction. In Japan there exist about 200 hospitals that can care childhood cancer arising about 2500 patients yearly. In 2013, with the aim of further integration and to provide

uniform accessibility to pediatric cancer treatment, the Ministry of Health, Labour and Welfare elected 15 core hospitals in seven regional blocks in Japan. Of these core hospitals,

the National Center for Child Health and Development (NCCHD) along with the National Cancer Center were selected as the two central organizations for pediatric cancer in 2014. In

Kanto-Koshinetsu area, we have about 700 to 750 pediatric cancers newly diagnosed per year.

Aim. The aim of this study is to evaluate the government medical plan in Kanto-Koshinetsu area for integration and dividing equally of childhood cancer patients.

Materials and methods. We collected total number of the pediatric cancers in 35 hospitals which belong to Liaison Council of Childhood Cancer in Kanto-Koshinetsu area and

provide the data through internet. The collected data were analyzed to reveal the change before and after the election of the core hospitals for pediatric cancer.

Results. Inpatient gross number (IGN) including newly-diagnosed and relapsed cancer patients in Kanto-Koshinetsu area was 5801 in 2013 in comparison with 5138 in 2010–2012,

and IGN of the four core hospitals was 2016 (34.8 %) in 2013 in comparison with 1705 (33.2 %) in 2010–2012.

For brain tumor, the number of patients treated in the top 10 hospitals, including 4 core hospitals, that cared more than 5 brain tumors in 2013 was 83.5 % of the whole (183/219)

and 10 hospitals in Kanto-Koshinetsu area did not give medical treatment of brain tumor. Similarly, for solid tumor, the number of patients treated in top 13 hospitals which cared

more than 10 patients of solid tumors was 77.5 % (299/386) and 5 hospitals did not treat solid tumors. In contrast, all 35 hospitals treated one or more patients of hematological

malignancies, and the number of patients in top 10 hospitals was 55.9 % (254/454).

In four core hospitals of Kanto-Koshinetsu area, the ratio of inpatient hospital total number of days before and after the election of core hospitals was decreased in hematological

malignancies, but increased in solid tumors and brain tumors.

Conclusion. Integration of the patients with brain tumors or solid tumors and dividing equally in hematological malignancies is realizing in Kanto-Koshinetsu area after the election

of 15 core hospitals in Japan.

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The positive impact of the protocols of empirical anti-infective therapy

on the drug resistance index (DRI) and pharmacoeconomics

V.P. Pirumova, G.G. Solopova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: antibiotic resistance, antimicrobial stewardship, pediatric heamatology/oncology

Introduction. Multidrug resistant organisms (MDRO) are the emerging problem across the world, and one of the most eff ective and mandatory steps in solving it is optimization

of antibiotics use. Here we present the fi rst results of the fi ght against MDRO at the Dmitry Rogachev Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia

(Center).

The Infection Prevention and Control Department (IPCD) has made a number of steps to rationalize the use of anti-infectives

Aim. Here we present the fi rst results of the fi ght against MDRO at the Dmitry Rogachev Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia (Center)

Materials and methods. On January 1st, 2014, the algorithm for empirical anti-infective therapy in febrile neutropenia was implemented. It included recommendations for fi rst

line (empiric) antibacterial therapy (in accordance with the patient’s risk group), spectrum of recommended evaluation tests and rules for treatment modifi cation. For example,

fl uoroquinolones and ceftazidime were excluded from empiric therapy and reserved for the targeted one only. The same recommendations were made regarding tigecycline and

ertapenem. Another step was the implementation of guidelines for perioperative antibiotic therapy, with cephalosporins I-II regarded as the drugs of choice in most of cases. Along

with those measures, precise control of antibiotics and antifungals discontinuation was established. For the evaluation of the eff ectiveness of the results we have calculated the use

of the most common classes of antibiotics and antifungals (per 100 bed-days)—before the intervention and a year after it. Microorganisms’-wise drug resistance index (DRI) was

also calculated.

Results. Having compared the respective rates before and after the intervention, we detected the overall decrease of use in vancomycin (64 %), piperacillin/tazobactam (15 %),

aminoglycosides (15 %) and fl uoroquinolones (7 %) in all the Center’s departments. Calculated separately, the rates were signifi cantly higher for the intensive care unit, where

interaction compliance with the IPCD was the highest: reduction of aminoglycosides use – 58 %, fl uoroquinolones – 66 %, cephalosporins III–IV – 8 %, vancomycin – 82,5 %,

piperacillin/tazobactam – 52 %, carbapenems – 20 %, linezolid – 47 %. The use of colistin remained on the same level, while the use of tigecycline raised 35 %.

As for antifungals, the analysis showed a 75 % reduction of use in amphotericin B and an 18 % reduction in case of voriconazole. Posaconazole raised 25 %. Although echinocandins

were used as the drugs of choice in empiric antifungal therapy in febrile neutropenia algorithm (in accordance with the ECIL recommendations), their use remained on the same level.

During the surveillance period DRI levels for P. aeruginosa and E. coli dropped down.

In terms of money, the reduction of anti-infectives use have saved the Center around €500 000 in 12 months. It is important to mention that the rates of infections-related mortality

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has not increased since the intervention (26 in 2013 compared to 16 in 2014).

Conclusion. The reduction of antibiotics and antifungals use and as a result – the decrease of microbial resistance and money savings in hematology/oncology hospital is an

achievable goal.

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Incidence, structure and ethnicity of solid tumors in children of the Republic of Dagestan

V.M. Delyagin1, D. Chanavova2, M. Madjidov2 1Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia;

2Dagestan State Medical Academy, Makhachkala, the Republic of Dagestan

Key words: tumors, incidence, structure, ethnicity, children

Introduction. The investigations of the incidence and ethnic distribution of malignant neoplasms (MN) in diff erent regions of Russia are required.

Aim. To investigate the incidence, structure and ethnic characteristics of MN in children in the Republic of Dagestan.

Materials and methods. According to the data from the Republic Oncology Dispensary and Republic Multi-fi eld Hospital 405 children and 92 adolescents with solid MN were

detected over period of 11 years.

Results. In the Republic of Dagestan an overall incidence of solid MN is 4.6–4.9 per 100 000 children. In standardized ratios the incidence of bone MN in diff erent years is 0.4–0.9,

in adolescents is 1.2–1.86 per 100 000 children. In the structure of the incidence brain tumors (30.0 %) are on the 1st place, then there are bone tumors (14.9 %), tumors of kidneys

(13.3 %), soft tissues (10.9 %), genital organs (6.6 %), eyes (5.4 %). Among the patients with bone tumors there was a predominance of children aged 9–14 (58.1 %), tumors of

soft tissues and eyes were more frequently registered in children of 1–4 years (33.3–77.8 % in diff erent tumor variants). In the investigated population tumors of lower limb bones

were registered more frequently (68.9 %), then there were observed tumors in axial skeleton bones (18.9 %), less frequently there were reported tumors in higher limb bones (12.2 %).

While within the structure of mortality of MN bone tumors in children were on the 3rd place (8.6 %) after hemoblastosis (55.9 %) and central nervous system (CNS) tumors (11.5 %),

bone tumors in adolescents took the 2nd place (14.5 %) as a cause of death in the overall mortality rate of MN. In case of the most common MN the children’s age is diff erent. CNS

tumors were more frequently detected in children aged from 9 to 14 – 46 (30.9 %), soft tissues MN – in children of 1 to 4 years – 18 (33.3 %), kidney tumors generally were in children

aged from 1 to 4 – 34 (51.5 %), 43 (58.1 %) cases account for children of 9–14 years in case of bone involvement. Less common tumors are: MN of eyes in children of 1 to 4 years –

21 (77.8 %) cases, all large intestine tumors account for the age of 9 to 17 years. In CNS tumors there were children of the Avarian ethnic group – 39 (26.2 %), 31 (20.8 %) children

from the Kumik ethnic group, 26 (17.4 %) children of the Darghin ethnic group, 23 (15.4 %) of the Lezghian ethnic group and the least number of native nationalities accounted for the

Lak ethnic group – 12 (8.1 %). Among the patients with soft tissue tumors there is a predominance of the Avarian ethnic group – 22 (40.7 %) children, then there are representatives

of the Darghin ethnic group – 15 (21.8 %), the Lezghian ethnic group – 5 (9.3 %). There were 5 (9.3 %) patients of the Kumik and 1 (1.9 %) patient of the Lak ethnic groups.

As in case of the previously stated localizations in kidney tumors there were more children of the Avarian ethnic group – 19 (28.8 %), then of the Lezghian – 15 (22.1 %),

the Kumik – 13 (19.7 %), the Darghian – 10 (15.2 %), the Lak – 1 (1.5 %) ethnic groups. Bone tumors are also generally represented by the Avarian ethnic group – 22 (29.7 %),

the Darghian – 16 (21.6 %), the Kumik – 12 (16.2 %), the Lezghian – 10 (13.5 %), the Lak – 4 (5.4 %) ethnic groups. Eye tumors are generally represented by the Darghian ethnic

group – 9 (33.3 %), 7 (25.9 %) of the Avarian, 6 (22.2 %) of the Kumik, 3 (11.1 %) of the Lak ethnic group and 1 (3.7 %) of the Lezghian ethnic group. In case of other localizations

children generally were of the Avarian ethnic group – 32 (33.7 %), the Darghian – 21 (22.1 %), the Lezghian – 15 (15.8 %), the Kumik – 9 (9.5 %) and the Lak – 6 (6.3 %)

ethnic groups.

Conclusion. MN incidence ratios are very similar in Dagestan, Moscow oblast and across the country in general. Further investigations of ethnicity of diff erent MN are required.

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The role of positron emission tomography in staging and determining treatment tactics

in children with sarcomas R.R. Bayramgulov1, I.I. Badrtdinova1, O.N. Lipatov2, D.A. Sinilo1

1Republican Children’s Clinical Hospital, Ufa, the Republic of Bashkortostan; 2Bashkirian State Medical University, Ufa, the Republic of Bashkortostan

Key words: sarcomas, children, positron emission tomography/computed tomography with 18F-fl uorodeoxyglucose, diagnostics

Introduction. Positron emission tomography/computed tomography with 18F-fl uorodeoxyglucose (PET/CT with 18F-FDG) is a prospective approach to diagnosis of sarcomas.

Investigations concerning the use of PET/CT with 18F-FDG for diagnostics of sarcomas in pediatric population are limited.

Aim. To evaluate the impact of PET/CT with 18F-FDG on staging and treatment planning in children with sarcomas.

Materials and methods. At our centre during the period 2014–2015, 26 PET/CT with 118F-FDG imaging exams were performed for 16 children (girls – 7, boys – 9; all children

are at the age of 0–18 years old) with histologically confi rmed diagnosis “sarcoma” (osteosarcoma (n = 4), Ewing sarcoma family of tumours (n = 4), synovial sarcoma (n = 4),

rhabdomyosarcoma (n = 3), sarcoma of kidney (n = 1)). The results of PET/CT with 18F-FDG were compared with the results of the standard visualization techniques, such as

ultrasound investigation, CT, magnetic resonance imaging and bone scintigraphy which are applied in accordance with international standards for diagnosis of sarcomas in children

for optimization of therapeutic approaches. The level of involvement was evaluated based on the analysis of PET/CT with 18F-FDG scans, on the analysis of fi ndings obtained by

standard visualization techniques only as well as on the fi ndings of PET/CT with 18F-FDG and standard visualization techniques performed simultaneously. The inclusion criteria for the

investigation were the following: the availability of protocols and medical images (standard visualization techniques, PET/CT with 18F-FDG), the results of morphological examination.

The period of the investigation was limited to the period of follow-up after the performed investigations (median follow-up duration – 12 months).

Results. PET/CT with 18F-FDG and standard visualization techniques were equally eff ective for the diagnosis of primary tumours (in 100 % of cases). PET/CT with 18F-FDG was

more sensitive than standard visualization techniques in case of the involvement of bones (sensitivity – 92 % and 58 % respectively) and lymph nodes (sensitivity – 94 % and

25% respectively) whereas CT defi ned lung metastases more accurately (sensitivity – 100 % and 27% respectively). The analysis of patients showed better results in the context

of simultaneously performed PET/CT with 18F-FDG and standard visualization techniques. Proper treatment tactics was accepted in 91% of cases, and this rate was higher when

compared with standard visualization techniques (60 %).

Conclusion. Use of PET/CT with 18F-FDG in sarcoma staging in children gives us important additional information about the extent of tumour process, and thus has a great impact on

further treatment tactics in case of performing PET/CT with 18F-FDG and standard visualization techniques simultaneously.

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A B S T R A C T N O . : P P - 1 6 4

Eye tracking method as a diagnostic tool for assessment of oculomotor control

and visual perception in patients with medulloblastoma

M. Shurupova1, V. Anisimov2, I. Borodina2, A. Latanov1, V. Kasatkin2 1M.V. Lomonosov Moscow State University, Russia;

2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: eye tracking, medulloblastoma, oculomotor control, eye movements, cognitive functions

Introduction. The cerebellum takes part in all classes of eye movements and gaze fi xation. It is known that cerebellar lesions of diff erent genesis cause range of oculomotor and

related cognitive disorders. Medulloblastoma spreads to vermis including regions which are mostly involved in the control of saccades but also contributes to smooth pursuit and

vergence (dorsal vermis, ansiform lobe, fastigal nucleus).

Aim. Nowadays eye movements are mainly recorded by eye tracking systems which are non-invasive and provide wide range of oculomotor parameters available to explore. We have

extended and adapted a number of paradigms which are applicable for investigation into oculomotor and cognitive functions.

Materials and methods. Three patients with medulloblastoma participated in our pilot study (1 male, 2 female; median age 14.5 years, range 13–17). All of them had a lesion of

the cerebellar vermis, 2 tumors spread to the 4th ventricle, 1 – to the hemispheres of the cerebellum. Each of the patients had his own stage of medulloblastoma (1 – M0, 1 – M1,

1 – M2). All of them underwent surgery (2 patients had total resection, 1 – subtotal) and received RT and/or CHT by protocol HIT.

The study consisted of two experimental sessions which were perfomed twice (at their fi rst and second visiting to rehabilitation center) to reveal the dynamic of oculomotor and

cognitive condition. Eye movements were recorded by Arringtion 30/60 Hz.

We applied six diff erent tests which included qualitative and quantitative criteria: gaze control test, gaze-evoked saccade test, “10 dots”, “fence”, “antisaccades”, “go/no-go”.

Results. Tests “antisaccades” and “go/no-go” didn’t reveal signifi cant diff erences between sessions probably because of low number of data. Gaze control test revealed the signifi cant

increase in gaze stability control in all patients in the second session compared with the fi rst because of the decrease in the number of intrusive saccades. One patient showed

signifi cantly increased accuracy of saccades in gaze-evoked saccades test. Test on strategy of scanning and working memory (“10 dots”) improvement of parameters such as search

time and number of fi xations in two patients. Finally, one patient executed visual rhythm saccade test (“fence”) rightly only in the second session.

Conclusion. We applied a range of qualitative and quantitative paradigm and revealed the improvement of oculomotor and cognitive functions after a period of a rehabilitation

which could characterize the condition of visual pathways and attention. We plan to increase the number of patients in our study to verify the succeed application of diagnostic tests

in clinical practice. Primary results suggest further fi ndings to design correctional paradigms for training impaired functions based on biofeedback.

A B S T R A C T N O . : O P - 2 4 7

Adenovirus hepatitis. Diagnostic features and diff erences

of tumor formation and metastasis. Case report

A.P. Shcherbakov, G.V. Tereshchenko, T.A. Komarova, D.M. Konovalov, D.S. Abramov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: liver, hepatittis, adenovirus, metastaes, livertumor

Introduction. Today one of the most dangerous complications of chemotherapy is the attachment of secondary infections. Main place of development of secondary complications -

pulmonary tissue that is associated with the process of the spread of secondary agents. With the development of systemic disease develops defeat parenchymal organs such as the

liver and spleen. One of the most rare and dangerous diseases is the appearance of virus-mediated liver infl ammation that leads to the death of most of the patient. Among all of viral

hepatitis in the world literature there is no detailed descriptions of the current X-ray pictures of the adenoviral hepatitis.

Aim. Presentation of radiographic changes of the liver parenchyma of confi rmed clinical cases of hepatitis adenovirus according conducted laboratory and morphological studies.

And also reveal a number of distinctive features that allow for a diff erential diagnosis between tumors and metastatic processes in the liver parenchyma.

Materials and methods. In the center of Pediatric Hematology, Oncology and Immunology, was conducted a comprehensive survey of children in post-transplant period over the

course of hemoblastosis (acute myeloid leukemia, aplastic anemia, acute lymphoblastic leukemia) with evidence of current infection. All the patients had performed blood cultures

for sterility, taken overall and biochemical analysis of blood, as well as research conducted MSCT of the chest and abdominal with the use of bolus contrast. The study was conducted

on 16-slice machine GE Brightspeed increments Scan 0.625 mm. Contrasting was calculated at the rate of 2 ml/kg. It was identifi ed during an initial infl ammation in the parenchyma

of both lungs, which was regarded as the mixed etiology (viral-bacterial). The blood tests showed an increase in value gradually liver enzymes (ALT, AST), in blood was detected

adenovirus. This was followed by repeated X-ray examination, which revealed the presence of liver parenchyma decreased contrasting areas of irregular shape with sharp contours,

loosely accumulating contrast agent in comparison with the surrounding parenchyma is not altered. It edged phase scan performed for 7 minutes after the contrast, reveals a gradual

fi lling of these pathologically identifi ed areas of contrast agents. Later in the analysis of biopsies of these areas have been identifi ed for acute infl ammation caused by adenovirus.

Results. During the studies were formulated diagnostic criteria specifi c to the current adenovirus Hepatitis - sharp jagged contours, lack of active contrast, the absence of the

peripheral rim of contrast, the gradual accumulation of contrast agent to the delayed phase scan, uniformity of structure. With help of this data can be more likely to diff erentiate the

acute viral hepatitis (according to clinical picture – adenovirus) with tumors and liver metastases in children.

Conclusion. It was fi rst detected X-ray picture of the current adenovirus hepatitis in children, as well as the data obtained for the diff erential diagnosis of such a complex and life-

threatening complications of chemotherapy.

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A B S T R A C T N O . : P P - 2 8 6

The results of serologic and bacteriologic examinations of bronchoalveolar lavage

for fungal infection in immunocompromised patients

S. Kondaurava, O. Aleinikova, I. Lukyanenko

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: bronchoalveolar lavage, fungal infection, immunocompromised patients

Introduction. Invasive fungal infections (IFI) as infection complications are common in immunocompromised patients. In case there are focal changes in lungs, bronchoalveolar

lavage (BAL) is carried out to clarify the etiology of disease. The fungal antigen galactomannan is specifi c for fungi class of Aspergillus. The detection of the galactomannan in biological

fl uids (blood, cerebrospinal fl uid, bronchoalveolar fl uid, pleural fl uid) may indicate invasive aspergillosis.

Aim. Serologic and bacteriologic examinations of bronchoalveolar lavage for fungal infection in immunocompromised patients.

Materials and methods. Twenty two BAL procedures have been carried out to detect infectious pulmonary diseases in 22 patients over the period of April, 2014 – December, 2015

in the Belarusian Research Center for Pediatric Oncology, Hematology and Immunology.

Results. The microbial agent was revealed in 6 cases (27 %). Four cases of bacterial and 2 cases of fungal infections were confi rmed (Мucor spp., Aspergillus fumigatus). In 8 cases (36 %)

the samples were contaminated by oral fl ora. In 6 (40 %) samples of bronchoalveolar fl uid out of 15 tested for galactomannan the result was positive (the result was considered

positive when the index was ≥ 1.0). In 1 case (17 %) of 6 positive results of galactomannan in BAL the fungal infection was proved. IFI was confi rmed in none of 9 cases of negative

galactomannan in BAL. Level of galactomannan both in blood and BAL was tested in 8 patients. Positive results of blood and bronchoalveolar fl uid galactomannan matched in

1 case. Invasive fungal infection in this case was not proved. In case of lungs IFI caused by fungi class of Мucor, the level of blood galactomannan was 0.17 (negative), galactomannan

in BAL was not tested. In case of lungs IFI caused by fungi class of Aspergillus, the level of blood galactomannan was 0.18 (negative) and galactomannan in BAL was 5.8 (positive).

Conclusion. BAL is an optional diagnostic test for identifi cation of etiology of lower respiratory tract infections. BAL helps a clinician to diagnose and reveal the etiology of infections

and initiate appropriate treatment in time. In our study BAL had signifi cant negative predictive value. However, BAL procedure is associated with certain risk of complications.

Contamination of bronchoalveolar lavage fl uid by oral fl ora is common. False-positive result of galactomannan level in BAL can confuse a clinician and lead to unnecessary antifungal

therapy. There is need of further research of BAL value in IFI detection.

A B S T R A C T N O . : O P - 3 0 7

The use of multislice computed tomography for the diagnosis of minimally invasive fungal

pneumonia in children with hematologic malignancies

A.P. Shcherbakov, G.V. Tereshchenko, G.G. Solopova, V.P. Pirumova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: fungal pneumonia, MSCT, ALL, AML, bone marrow transplantation

Introduction. Modern chemotherapy has recently made great strides in the issue of the treatment of hematological malignancies. For their treatment used chemotherapy drugs,

hormones, and bone marrow transplantation. In recent years signifi cantly increased the life expectancy of these life-threatening diseases. The most dangerous complication of the

treatment at the moment is the addition of secondary infections and the development of life-threatening infl ammation. Being in hematopoiesis aplasia or in a state of induced

neutropenia, the body can not adequately resist bacteria, viruses and fungi. It is the development of uncontrollable fungal pneumonia and subsequent fungal sepsis is one of the fi rst

places among all secondary pathological processes associated with the process of the treatment of children with acute lymphoblastic and myeloblasts leukemia, lymphoma, aplastic

anemia.

Aim. Presentation of the results of the diagnostic study.

Materials and methods. To all patients in the induced aplasia of hematopoiesis with continue fever for 48 hours without any signs of the primary infection locus was performed chest

computer tomography. The study was made on GE Brightspeed computer tomography with scan increments of 0.625 mm. If the native study identifi ed major foci of infi ltration of lung

tissue located in the lung parenchyma, was performed the bolus contrasting using iodinated contrast. The dose of contrast agent was calculated at 1/1.5 mL/kg. Part of the research

was performed using general anaesthetic. in subsequent There was performed bronchoscopy with taking of bronchoalveolar lavage for the indication of the morphology of the

pathogen if any changes in the lung parenchyma were found. In the future, the results were discussed in the framework set up an interdisciplinary working group to study the fungal

disease of the lungs. We investigated 450 children (220 boys, 230 girls, average age 3.5 ± 0.5 years) with diff erent leucosis’ variants (acute lymphoblastic and myelogenousleukemia)

that received specifi c therapy according to chemotherapeutic protocols (ALL-MB-2008, AML-MM-2006).

Results. There were detected x-ray signs of Invasive fungal infections (IFI) at earlier stage of infection in 90 % of children. Classic halo sign (97.5 %), segmental or subsegmental

consolidation (75 %), single or multiple nodules (70 %), nodular infi ltrates (15 %) were considered as early indicators of IFI.

Nearly half of patients had positive galoctomannan (≥ 0,5) and mannan (≥ 1) in BAL.

Conclusion. HRCT can detect invasive disease at an earlier stage of infection. Early and frequent CT scanning has been used to direct BAL galactomannan antigen detection and has

shown good sensitivity prior to instigation of empirical therapy. Only an interdisciplinary approach to the problem of invasive pulmonary aspergillosis can achieve maximum results

in the treatment of fungal infections in children.

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A B S T R A C T N O . : P - 3 8 7

The frequency of detection of herpes group viruses

in children with oncohematological diseases

Y.Y. Kozel, I.B. Lysenko, V.V. Dmitrieva, T.A. Zykova, O.V. Kozyuk, E.E. Pak

Rostov Scientifi c Research Institute of Oncology, Rostov-on-Don, Russia

Key words: herpes group viruses, oncohematology in childhood

Introduction. The infectious complications are one of the frequent reasons of a fatal case in children with oncohematological diseases. The infections caused by the representatives

of the herpesviruses family are worth of a special attention, their activation is possible on the application of the current high dose chemotherapy methods.

Aim. To investigate the prevalence of the herpes group viruses in children with the oncohematological diseases.

Materials and methods. The study was performed on the basis of the Department of Hematology of FSBU RSRIO. 16 patients with Hodgkin lymphoma (HL), 14 patients with

non-Hodgkin lymphoma (NHL), 18 patients with acute leukemia (AL) were included in the investigation. The total amount of the investigated patients is 48, aged from 5 months

to 18 years, the median is 6.2 years. The samples of a whole blood were investigated. An extraction of the DNA of a herpes simplex virus 1, 2 (HSV-1, 2), cytomegalovirus (CMV),

Epstein-Barr virus (EBV) and human herpesvirus 6 (HHV-6) was performed by method of a sorption on the magnetic particles in an automatic mode with MagNa Pure Compact Nucleic

Acid Isolation Kit (Roche, Switzerland), as well as in a manual mode sorption method on the silica gel particles with “AmpliPrime DNA-sorp-V” (OOO “InterLabService”, Moscow) kit.

The DNA detection and quantitation was performed by multiplex polymerase chain reaction (PCR) with hybridization-fl uorescence detection of amplifi cation products on Rotor Gene

6000 thermocycler (QIAGEN, Germany) with “AmpliSens HSV1,2-FL” and “AmpliSens EBV/CMV/HHV6-screen-FL” of Rospotrebnadzor (The Federal Service for Supervision of Consumer

Rights Protection and Human Well-Being) CRIE production.

Results. A genetic material of the diff erent herpesviruses was found in 27 patients in 56.3 % cases. The most frequent was DNA EBV – in 13 (48.1 %) patients. DNA HHV-6 was

reported in 40.7 % cases (11 patients). DNA CMV in 2 patients – 7.4 %. DNA HSV-1, 2 was reported in 1 (3.7 %) patient. Mixed-infections were registered in 5 (10.4 % of a total amount

of the investigated patients and 18.5 % of the infected patients). The infection by 3 viruses simultaneously (CMV, EBV и HHV-6) was reported only in 1 case, 2 viruses were in the other

cases. Predominantly it was a combination of EBV and HHV-6 (66.7 %). One patient was infected simultaneously by CMV and EBV, 3 patients were infected by CMV and HHV-6. The

maximum incidence belongs to EBV: 18.5 % were in patients with NHL and AL, 11.1 % were in patients with HL. HSV-6 is defi ned in 18.5 % patients with AL, in 14.8 % patients with

NHL and in 7.4 % patients with HL. CMV is less defi ned: 3.7 % in patients with NHL and AL. 1 patient with HL was infected by HSV-1, 2 – 3.7 %. The mean ratios were from 1.2 to 1.8,

ratio range – 0.6. The maximal ratios of viral load, %: from 2.1 to 5.1, range was 3 units. The viral load level did not exceeded 2.1 lg copies per 105 leukocyte for HSV-1, 2; 2.5 – per

105 leukocyte for CMV; 2.8 lg for EBV and 5.1 lg for HHV-6.

Conclusion. In the course of herpesviruses prevalence investigation in patients it was reported that 56.2 % have infection at the diagnosis of malignant disease. The mean patients

age was 6.2 years. A detection of CMV and HSV-1 happened far less and was 7.4 % and 3.7 %, respectively. Mixed infections were registered in 18.5 % patients of the amount of

infected patients. Considering high herpesviruses prevalence in children, a high agent activation prevalence in an immunosuppression the evaluation of the viral load level signifi cance

at diagnosis of infections caused by herpes group viruses is necessary.

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A B S T R A C T N O . : O - 1 3 0

Musculoskeletal sequelae of childhood solid tumours survivors, treated with intensive

chemotherapy, surgery and radiation therapy

A. Petrichenko, E. Bukreeva, N. Ivanova

Applied Research Center for Specialized Medical Care of Children named after V.F. Voyno-Yasenetsky, Russia

Key words: solid tumours survivors, musculoskeletal late eff ects

Introduction. Advances in diagnosis and treatment of childhood cancer have dramatically increased long-term survival and it is now evident that the disease and its treatment can

signifi cantly impair long-term health. Childhood solid tumours survivors are known to be at risk for the serious musculoskeletal late eff ects that may result in disability

Aim. Recommendations for screening, prevention, and management of survivors and the evaluation of effi ciency of cancer rehabilitation of children, treated with intensive

chemotherapy, surgery and radiation therapy.

Materials and methods. 102 children and adolescents with solid tumours were treated between 1987 and 2014 years. The mean age at the date of orthopedic diagnosis was

13.25 ± 0.43 years (from 2 till 24 years). Common sites of primary disease include the thorax, abdomen, trunk and extremities. Most often the aff ected area was the area of the lower

extremity – 48.0 % of the cases. According to the histological variation of the tumor 40.2 % of the patients had morphologically confi rmed diagnoses of Ewing sarcoma, 28.4 % – osteosarcoma,

31.4 % – other solid tumours. 31.0 % patients had primary distant metastases. Among them, 46.9 % patients had solitary metastases, 53.1 % patients had multiple metastases.

Treatment consisted of neoadjuvant chemotherapy, the radiotherapy of the initial tumor and metastasis left after the induction and/or oncologic surgery and adjuvant chemotherapy.

The local control of the tumor consisting of the surgical ablation of the primary lesion and metastases, if the technical opportunity of this stage is available, including limb-sparing

procedures. The most common late eff ects we had observed were: scoliosis – in 78.4 % cases, muscular hypoplasia – 74.5 %, osteopenia – 55.9%, limb-length discrepancy in spite of

usage of growing endoprosthesis – 45.1%, poor joint movement – 66.7%, musculoskeletal deformity – 39.2%. 77.5% patients had from 1 till 5 late eff ects, 22.5% – 6 till 11. We used

the NCI Common Terminology Criteria for Adverse Events (CTCAE) for reporting. We have not observed serious Adverse Events, like Grade 4: life-threatening consequences and Grade 5:

death related to Adverse Events. Among the children who developed the largest number of eff ects, children who received СT and radiotherapy were 17.4 %, in the group with major

surgery – 56.5 %, CT, surgery and radiotherapy – 26.1 %. This indicate, that some researchers revaluate the role of radiation therapy in the development of musculoskeletal sequelae.

All long-term survivors of childhood cancer should attend rehabilitation therapy, because it is dramatically increase the quality of life. An individual rehabilitation program consist

with combined early mobilization, physical exercise, kinesiotherapy, aquatic rehabilitation and orthopaedic correction, laser therapy, massage, gait training.

Results. Currently 90 patients are alive without disease, following up of 12 to 343 months. We have observed Grade 2 Adverse Events (CTCAE) in 43.3 % cases. The lowest grade by

Musculoskeletal Tumor Rating Scale we have observed in cases with distal leg localization – 51.4 %.

Conclusion. Interdisciplinary team, provide services that enable patients to achieve their highest functional status to permit them to return to their role in society. We suggest that

the usage an individual rehabilitation program can dramatically increase the quality of life. Long-term survival is possible, even for patients with metastatic disease.

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A B S T R A C T N O . : P - 1 4 9

Secondary tumours in children of the Nizhny Novgorod region

L. Bogdanova, L. Privalova, V. Alexeeva, K. Isaev, E. Abramova, O. Kozhakova, L. Karaseva, A. Alekseev

Nizhny Novgorod Regional Children Clinical Hospital, Russia

Key words: secondary tumours, children, haematological malignancies, Hodgkin lymphoma, radiotherapy

Introduction. In recent years there has increased a number of follow up reports on children, who received complex therapy of malignancies and were cured. The problem has an

important social aspect, since the therapy has a signifi cant eff ect on the life quality of both: the patient himself, and his off spring. Secondary tumour (ST) development in such patients

is a new burning problem, and according to diff erent authors, the frequency of ST varies from 2 to 12 %. There are synchronous tumours, which occur promptly after the fi rst lesion

detection, and ST induced by chemoradiotherapy. ST risk depends signifi cantly on specifi c therapy intensity, patient`s age at the moment of treatment, gender, as well as the time

period passed after the treatment termination

Aim. The investigation aimed at studying the frequency and peculiarities of the course of ST in children in Nizhny Novgorod region.

Materials and methods. During the period from 1989 to 2015, 12 patients with ST were treated in the Oncology Department of Nizhny Novgorod Regional Children Clinical Hospital.

Results. 1) ST occur more frequently in children over 9 years old (92 % observations), and only one child aged 6 years; while primary tumours develop mainly in children under

10 years (70 %); 2) boys prevailed among the patients (60 %); 3) solid ST developed in two children having been treated for solid tumours; 4) most frequently, ST are recorded

in patients with haematological malignancies (total 80 % observations), among them, 40 % patients had had Hodgkin’s lymphoma (HL), and 30 % patients – acute lymphoblastic

leukemia (ALL). It is interesting to note that all children treated for ALL appeared to have ST, and three children with HL had haematological malignancies (non-Hodgkin lymphoma

or leukemia), and one child – gastric cancer; 5) tumour response in patients under study was from 2 months to 16 years. The younger a child was at the moment of primary tumour

detection, the longer the latent period was before the ST developed: in our study in the children under 7 years (50 %) it was from 6 to 16 years; and in children over 7 years (50 %) it varied

from 2 months to 2 years, and only in one case the patient was a 6-year-old child; 6) radiotherapy is of great importance in the development of ST: among the patients treated, only

one appeared to have had no radiotherapy during the fi rst disease due to an early onset of the ST (when the fi rst disease had not yet been treated completely). There can be traced

the dependence of the remission period on a dose and the number of radiation areas during the fi rst disease: the higher the dose and more the number of areas, the shorter the latent

period of the ST; 7) the therapy intensity of the fi rst tumours infl uences the growth of ST: 50 % children received therapy for lymphoma and ALL; 8) a latent period for secondary

hemoblastoses lasted, mainly, from two months to 6 years (median 2 years 3 months), while for solid ST it was from two to 16 years (median 7 years).

Conclusion. 1. Most frequently, ST are recorded in patients with haematological malignancies, among them 40 % patients are those with HL. 2. ST are induced mainly by radiotherapy,

the doses and the number of radiation areas being of importance. 3. There is the dependence of a latent period time before the ST development on the primary tumour type.

4. Currently, outcomes are signifi cantly better: in our study, 50 % children with ST have survived, the tumour response in all children being over 5 years.

A B S T R A C T N O . : P - 1 7 5

Recovery of motor skills in patients with malignant neoplasms

A.V. Baerbakh, E.V. Zhukovskaya, V.N. Kasatkin

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: rehabilitation

Introduction. For the last 40 years in Russia, the survival of children with cancer has increased at 60 % (V.G. Polyakov, 2015). This fact enhances considerably the relevance of

improvement of rehabilitation technologies for patients with oncohematological diseases. Many cancer survivors who had musculoskeletal disorders (soft tissue tumours, bone

sarcomas) require integrated/complex/comprehensive rehabilitation including recovery of movement/motor skills.

Aim. To develop and implement a protocol for diagnostics and correction of movement disorders in cancer survivors.

Materials and methods. During 2015, a computer force plate with biofeedback was analyzed. A study group included 9 patients from LRNC “Russkoe pole”: 3 patients with CNS

tumours, 4 children with acute leukemia, 2 children with bone sarcomas. The mean age of the patients was 9.6 ± 1.3 years old. All the children have been in remission for 2 years

and more. Children with bone sarcomas underwent treatment for malignant neoplasms: patient with osteogenic sarcoma, 1 patient with Ewing sarcoma, without amputation

and endoprosthesis replacement. A testing was performed before the therapy start as well as after the completion of training period, with multiple repetition of trainings with

biofeedback. The dynamics of functional impairment recovery was assessed separately for osteoarticular, muscular and nervous system. The improvement of the ability to keep

balance and the coordination of movement was accepted as the measure of treatment effi ciency.

Results. A total amount of trainings varied from 8 to 12. On average, the duration of training was 12-15 minutes. The maximal duration of training (20 minutes) was achieved in the

patients of senior age group without serious neurologic impairment. The main diffi culties while executing the protocol were observed in patients of the younger age group.

Conclusion. Stabilometrics improves dynamic and postural stability of a patient in an upright position; owing to multiple repetitions and biofeedback, the walking stereotype and

walking rhythm are formed. Rehabilitation events should be active and should be performed as early as possible. Timely multimodal rehabilitation for children off ers the possibility

to correct the consequences of disease and antitumour treatment, that would further reduce the disability status of patients. Use of robot-assisted gait training with biofeedback is a

promising trend in comprehensive rehabilitation of children and adolescents with CNS tumors and malignant neoplasms of other location.

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A B S T R A C T N O . : P - 2 5 9

Application of cosmetics in the rehabilitation of children

with skin manifestations of chronic GVHD

O. Rassokhina1, P. Trakhtman2, N. Potekaev1 1Pirogov Russian National Research Medical University, Moscow, Russia;

2Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: skin manifestations of chronic GVHD, rehabilitation

Introduction. Brisk growth of Hematology has led to survivability enhancement after transplantation of hematopoietic stem cells, which warrants an increase in the number

of patients with chronic graft-versus-host disease (GVHD). Cutaneous manifestations of chronic GVHD has an additional negative impact on the physical, social and psychological

rehabilitation of the patient.

Aim. To perform assessment of capability for cosmetics to achieve normalization of the skin.

Materials and methods. 15 patients aged from 3 to 24 years with chronic GVHD was examed: 6 (40 %) females and 9 (60 %) males, 6 patients (40 %) aged 3–5 y. 1 (6 %) 6–12y.,

5 (33 %) 13–17 y and 3 (20 %) 18–24 y.o. The morphological changes of the skin was performed using dermoscopy and ultrasound, the functional state of the skin by sebumetry

and corneometry. To determine changes in quality of life there was calculated a dermatological life quality index,estimation of the psycho-emotional status of patients was evaluated

based on: Hamilton and Beck scales for depression and Covi, Spielberger scales-for anxiety. The general condition of the patients was evaluated based on the scales of Lansky and

of Karnovsky. Assessment of the state was carried out before and during treatment. Primary survey of patients with chronic GVHD: decrease in the thickness of the epidermis for

all patients, an increase in the thickness and density of the dermis by ultrasound skin with 80 % of patients, changes in blood vessels in the nail bed dermoscopy with 30 % and

reduced moisture and greasiness of the skin for all patients.Patients examined with the help of psychotherapy scales had mild depressive disorder (8–10 points for Hamilton), anxiety

(1–2 points on a scale Covey). Dermatological quality of life index was (9–14.6 points).The general condition of patients according to the Lansky scale was 50 for 29 % of all patients,

80 for the remaining 71 %, under the Karnovsky scale 25 % of patients had 70, 12.5 % had 80 and 62.5 % had 90.To correct the dryness of skin emollients were used 2–5 times

a day, depending on the condition; hyaluronic acid was used as dermatoprotektor in combination with mucopolysaccharides. In order to accelerate epithelialization crack, reduce

infl ammation there was topically applied cosmetics containing panthenol, copper, zinc, manganese, thermal water.

Results. Rational use of cosmetics on background of standard immunosuppressive therapy resulted in a reduction of the subjective discomfort (regression feeling of tightness and

itching) for 80 % of patients, an increase in the level of moisture and greasiness for 93.3 % of patients. With the reduction of skin manifestations of chronic GVHD and improvement

of general condition, confi rmed by clinical, laboratory and instrumental data, psychoemotional state of patients has improved. Points on a scale of Hamilton decreased to 5, and on

a scale of Covey-0, which corresponds to the rules. DILQ decreased to 3 to 80 % of patients. The general condition of patients according to Lansky Scale was 50 for 14 %, 86 for 80 % of

patients, according to Karnovsky scale the results were 70 for 12.5 % of patients, 80 for 25 % of patients and 90 for 62.5 % of patients.

Conclusion. Applying cosmetics makes it possible to reduce skin manifestations of chronic GVHD, which improves the emotional state of patients and has a positive impact on

rehabilitation.

A B S T R A C T N O . : P P - 2 7 3

Assessment of gonadal function in girls after allogeneic transplant

with treosulfan-based conditioning regimens

L.I. Papusha, Yu.V. Skvortsova, E.Yu. Ilina, L.N. Shelikhova, M.A. Maschan,

D.N. Balashov, G.A. Novichkova, A.A. Maschan

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: ovarian function, treosulfan

Introduction. Gonadal damage occurs in about 100 % of recipient of allogeneic transplants with classic high dose busulfan – containing conditioning regimens. However, little is

known about gonadal function in recipients of new myeloablative regimens containing other alkylators. Treosulfan exerts powerful immunosuppressive and myeloablative action

when used in cumulative doses > 36 g/m2 while its visceral toxicity is modest, leading to its wide use as a backbone of conditioning regimens in wide range of hematological

malignancies as well as non-malignant conditions.

Aim. To evaluate ovarian function in girls who received treosulfan-based conditioning regimen.

Materials and methods. Gonadal function (puberty staging by Tanner, blood tests for luteinizing hormone, follicle stimulating hormone and anti mullerian hormone (AMH) was

evaluated in 20 females recipients of treosulfan-based conditioning regimens. The conditioning regimen consisted of treosulfan 42 g/m2, fl udarabine 150 mg/m2, melphalan 140 mg/m2.

Mean age at HSCT was 14.5 years (range 10–20), median age at time of the study 16 years (13–22).

Results. Eight patients, initially diagnosed with ovarian failure, showed recovery of ovarian function 6–12 months after HSCT. These girls had regular menstrual cycle, but serum

AMH levels were signifi cantly decreased (AMH 0.5 ± 0.2 ng/ml) comparing with the standart levels in all but two patients in this group. Spontaneous puberty and menarche occurred

in seven girls, who were prepubertal at the time of HSCT. Five patients developed hypergonadotropic hypogonadism (LH 35.55 ± 18.19 UI/l, FSH 98.65 ± 52.06 UI/l, estradiol

12.14 ± 6.85 pmol/l, AMH 0.13 ng/ml) and required hormone replacement therapy.

Conclusion. High dose treosulfan conditioning may allow prompt gonadal function recovery. Young women with diminished ovarian reserve should be informed about the risk of

premature menopause. Eff ective fertility preservation methods should be discussed with these patients.

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A B S T R A C T N O . : P P - 2 7 7

Study on late eff ects of treatment of oncological diseases in children:

the experience of a specialized clinic

M.E. Lokhmatova, A.E. Rudneva, E.E. Ilyina

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: children, acute leukemia, lymphomas, late eff ects, therapy program

Introduction. Modern therapy of oncological diseases in children is highly eff ective but remains quite toxic and has late side-eff ects. Children cured of oncological diseases need

a long-term systematic follow-up the aim of which is the control of disease remission maintenance and maximal early detection of the therapy late eff ects. The study on incidence and

expressiveness of late eff ects of the treatment is eased if a large cohorts of patients are formed that remain in contact with the specialized clinic and are thus available for systematic

examinations.

Aim. To arrange the data of pediatric patients that had diff erent oncological diseases.

Materials and methods. Since 2007 in FRSC PHOI n.a. Dmitry Rogchev the prospective data collection on health of patients after the treatment of oncological diseases is carried

out. From April, 2007 to December, 2015 765 patients aged from 1.5 to 30 years were registered (acute lymphoblastic leukemia – 44 %, non-Hodgkin’s lymphomas – 22 %, Hodgkin’s

lymphoma – 15 %, brain tumors – 4 %, soft tissue sarcomas – 3 %, neuroblastomas – 2 %, acute myeloid leukemia (AML) – 2 %, other malignant diseases – 7 %), the interval from

the end of the therapy is 1.5 months to 20 years (median – 3.25 years).

Results. Over the whole period of the follow-up 3366 present and absent encounters of patients were registered. 48 adverse events were noted: 32 (4.2 %) relapses of the main

disease, 13 (1.7 %) secondary tumors, 3 (0.4 %) patients died in remission at place of residence allegedly from infectious complications. The detection of relapses and secondary

tumors at scheduled follow-up before complaints was observed in 15 (47 %) cases of 32 and in 3 of 13 cases, respectively. Eight (17 %) of 48 adverse events were at the period of more

than 5 years after the end of the treatment: 1 relapse of anaplastic large-cell lymphoma and 7 secondary tumors. The mean term of the relapse development was 1.8 years (3 months,

Burkitt lymphoma – 5.8 years, ALK-lymphoma), secondary tumor – 5.5 years: (2.5 years, thyroid cancer after Hodgkin disease – 12 years, AML after rhabdomyosarcoma). Loss-to-

follow-up (the absence of information about patient for more than 12 months) was 32 % (246 patients), out of which 30 (12 % of lost and 4 % of the total number of patients) were

lost in the fi rst year after the treatment. More than 450 patients are regularly examined. The follow-up is carried out on individual plans that allow to detect such late eff ects as growth

arrest, hypothyroidism, emmeniopathy, postradiation fi brosis, mental retardation, obesity and others. At the moment due to the lack of resources in-depth cardiological, pulmonary

examinations are not carried out, reproductive health and the quality of life of patients are not studied.

Conclusion. In our country this work is the fi rst attempt to arrange the data of pediatric patients that had diff erent oncological diseases. With a large number of disadvantages

(high percentage of losses-to-follow-up, absence of in-depth study of some organs and systems) this group of patients can be a basis for a systematic study of late therapy eff ects,

particularly on therapeutic protocols that are unique to our country. The detection of adverse events in a later date indicates the necessity of a long-term follow-up.

A B S T R A C T N O . : P - 2 8 3

Functional and ultrasound diagnosis in the assessment of late toxicity in children with

oncological diseases

T.V. Kudinova, P.M. Gorbylev

Treatment and Rehabilitation Scientifi c Centre “Russkoe pole” of Federal Research Center of Pediatric Hematology,Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: late eff ects, functional and ultrasound diagnosis, leukemia, osteosarcoma, histiocytosis, hematopoietic stem cell transplantation, rehabilitation

Introduction. The problem of the development of late toxicity still remains unsolved, and timely diagnosis including ultrasound and functional methods would help to detect specifi c

changes as early as at the preclinical stage.

Aim. Considering the incidence rate of late complications after treatment of the above mentioned diseases, we have suggested the algorithm of functional and ultrasound studies.

Materials and methods. Neurotoxicity in patients with acute lymphoblastic leukemia develops in 43.6 % of cases and manifests itself as encephalopathy (with convulsive seizures

in 10 % of cases), polyneuropathy. Cardio- and hepatotoxicity develop in 20 % and 14 % of children respectively. It is recommended to place emphasis on electroencelography (EEG),

electroneuromyography (ENMG), electrocardiography (ECG) with Holter monitoring, echo-cardiography (Echo-CG), ultrasonography (US) of abdominal cavity, US of intestine and

lymph nodes. Brain stem tumors account for 10–20 % of all central nervous system (CNS) neoplasms. These aspects combined with the received therapy/being received, including

hormonal therapy, may cause respiratory, cardiovascular, hypothalamo-pituitary-adrenal systems defi cits. In addition to EEG monitoring and EKG, we recommend to estimate external

respiration function, perform abdominal US, as well as evaluate visual evoked potentials in case of sight impairment.

Late eff ects associated with hematopoietic stem cells transplantation are accompanied by the involvement of the liver, lungs (in the form of interstitial pneumonits/pulmonits), joints,

muscles, by the atrophy of lymphoid organs. In 40 % of cases, impairment of pancreatic function and dysfunction of reproductive system are detected, cataract is diagnosed in 20 % of

cases. Also, there is a risk of developing a brain tumor, a thyroid gland tumor and an osteosarcoma. The list of studies includes EEG, EKG, visual evoked potentials, external respiration

function (ERF), US of abdominal cavity organs, intestine, thyroid gland, all groups of lymph nodes, soft tissues.

In children with Langerhans cell histiocytosis, skeletal involvement is registered in 60–80 % of cases, lung changes – in 10–30 % of cases. Liver, spleen, lymph nodes pathology

is observed in 10–15 % of patients. CNS injuries exhibit local degenerative changes or mass lesion pattern. A protocol of examinations should contain EEG, evoked potentials, ERF

evaluation, ECG, Echo-CG, US of abdominal cavity organs, and peripheral lymph nodes.

According to the degree of tumor extension, 80 % of osteosarcomas in children may be divided into localized osteosarcomas, accompanied only by the involvement of bones with

adjacent muscles and tendons, as well as into metastatic ones with multiple involvement of bones, lungs (85 % of cases), brain and internals. Taking into consideration diff erent

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incidence of neuro, ortho, nephro and cardiotoxicity, we recommend to include into a protocol such examinations as: ECG with auditory evoked potential, ENMG, densitometry, ECG,

Echo-CG, ERF, US of abdominal cavity organs and kidneys, US of soft tissues and lymph nodes.

Results. Following the work that has been carried out, protocols of functional and ultrasound diagnosis of late eff ects in pediatric cancer survivors will be developed. Rehabilitation,

oncology and hematology may be the possible fi elds of its application.

Conclusion. Ultrasound and functional methods may be widely used in diagnosis of late toxicity in patients with oncology diseases. After conducting a defi nite volume of examinations

and analyzing examination fi ndings the suggested algorithm may be reconsidered and modifi ed.

A B S T R A C T N O . : O - 3 3 7

Microelemental homeostasis in patients with oncological diseases

V. Bondarenko, E.V. Zhukovskaya, Yu.A. Obukhov, L.V. Sidorenko, I.R. Minulin, B.V. Kholodov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: microelements, rehabilitation

Introduction. The interest in microelemental homeostasis in patients with malignant neoplasms is present both on a disease diagnosis stage and at the period of remission

establishment. There are many hypothesis about the applicability of the microelemental homeostasis study in patients with oncological diseases. However, none of the current

theories has an essential evidential base. The problem is mostly in a planning stage.

Aim. To evaluate the content of toxic and essential microelements in diff erent biological environments within a pilot study of patients that are on diff erent rehabilitation stages.

Materials and methods. 35 hair and blood/serum probes, 23 saliva probes were studied in 40 patients that were on a rehabilitation in Treatment and Rehabilitation Centre “Russian

fi eld” in 2015. Solid soft-tissue tumors were in 28 patients: nephroblastoma – 9, neuroblastoma – 6, bone sarcomas – 4, soft-tissue tumors – 3; leukemia and lymphomas were in

12 patients. The remission duration was 1 year and more. The age varied from 4 to 16 years. Probe testing was performed on 40 and more microelements with the help of an atomic

emission spectrometry with an inductively coupled argon plasma on Optima 2000 DV unit (PerkinElmer SCIEX, USA) and a mass-spectometry with the inductively coupled argon

plasma on Elan 9000 (PerkinElmer, USA) unit. A table of referential values that were developed earlier was used as comparative analysis data. (A.V. Skalnyi, 2006).

Results. A less number of deviations was detected in investigational blood samples: an increase of content of toxic and conditioned toxic chemical elements Al, Cd, Ag were shown

at 1–1.3 from referential values but there is a downtrend of content of essential chemical elements Са, I, Se, Cr. A decreased chrome content was shown in 9 of 10 patients that were

diagnosed with a metabolic syndrome, which conforms the regulation on an association of carbohydrate metabolism disorders with a chrome stress.

Main eff ects in saliva samples were multidirectional changes of essential elements content, their intensity both in respect of diversity and deviation values from referential values also

was defi ned by the severity of dental pathology.

Hair is a standard investigational substrate of microelemental structure and a relevant medical care is registered in a register of a CMI system. A content of microelements in hair

refl ects long-term trends of a homeostasis maintenance. Along with a silver and cadmium excesses, toxic and conditioned toxic elements excesses were not registered in patients.

Deviations in the content of calcium, molybdenum, manganese, selenium, zinc in 2 or more times were reported in hair in children of an investigational group.

Conclusion. Considering the fewness of investigational group and a diversity of received microelemental content changes of investigated biological environments, it is not possible

to defi ne the specifi city of microelemental deviations depending on a diagnosis and a received deviation.

According to data of performed studies it is possible only to state the fact of the presence of microelemental homeostasis deviations in children that were cured from malignant

neoplasms and were on a long-term remission.

At the process of data collection the signifi cance of microelementosis in oncohematological patients in remission and the necessity of their corrections are to be specifi ed.

A B S T R A C T N O . : O P - 3 3 8

The structure of the functional impairment in adolescent survivors of childhood cancer

H. Zbarouskaya

National Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Republic of Belarus

Key words: functional impairment, childhood cancer survivors

Introduction. Adolescent survivors of childhood cancer are at risk for medical and psychosocial sequelae that may adversely aff ect their health status. The problems of adolescent

cancer survivors appear to be distinct from both older adult and pediatric survivors. For purposes of this issue, we will address survivors as patients who have completed initial

treatment for cancer and who are without evidence of disease. Functional impairment after cancer and its treatment may potentially associated with limited opportunity for

participation in community or social roles.

Aim. To determine structure of functional impairment with special attention to diff erences between rural and urban inhabitants.

Materials and methods. Health status were assessed in 74 adolescent survivors of childhood cancer, aged 15–19 years (median 16.6 years), male/female ratio 1.55, from whom

51 were urban and 23 rural residents. The diagnoses were: hemablastosis 39 (52.7 %) cases (leukemias – 13 cases, lymphomas – 23 cases), solid tumors 35 (47.3 %) cases

(CNS tumors – 7, bone tumors – 11, soft tissue sarcomas and renal tumors – 4 each, retinoblastoma – 3, other solid tumors – 6 cases). Median time after diagnosis was 6.5 years,

range 1–15 years. We used International Classifi cation of Function, Disability, and Health (ICF) to determine limitations resulting from both medical and psychosocial sequelae.

Results. Among surveyed the most common consequences of childhood cancer and its treatment were endocrine, cardiopulmonary, neurocognitive and musculoskeletal

dysfunctions. We revealed endocrinopathy in 15 (29.4 %) urban and 6 (26.1 %) rural inhabitants (P = 0.76) with prevalence of obesity in those who live in towns – 8 (15.7 %) vs 0 (P = 0.04).

Musculoskeletal disorders were presented in 6 (11.8 %) urban and 4 (17.4 %) rural citizens (P = 0.5), sensory, cardiac and pulmonary organ system dysfunctions in 7 (13.7 %)LATE

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and 3 (13 %), 4 (7.8 %) and 1 (4.3 %), 3 (5.9 %) and 1 (4.3 %) teens, respectively (P = n.s.). 9 (17.6 %) from urban residents were receiving secondary or higher education, compared

with none from rural locality (P = 0.03).

Conclusion. In developing rehabilitation interventions for childhood cancer survivors we must take into account their functional impairment and place of living to enhance their

social participation.

A B S T R A C T N O . : P P - 3 4 4

Dental health of the patients cured of malignant neoplasms in childhood

Yu.A. Оbukhov, Е.V. Zhukovskaya, A.V. Tushevskaya, L.V. Sidorenko

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: stomatology, leukemia, children

Introduction. Cancer treatment in childhood aff ects the tooth development by direct toxic eff ects on odontogenic cells, or on irregulations pathways of signalling interactions

between ectoderm and mesenchyme. Proliferating preodontoblasts and their precursors generally have a higher sensitivity to unfavourable factors in comparison to the functioning

mature cells (I. Thesleff , 2003). Mechanisms of the infl uence of antitumor therapy resulting in stomatological complications remain considerably unexplored (P. Kanchan et al., 2014).

In addition, according to the data from a Childhood Cancer Survivor Study in a long-term period only 60.4 % of 9434 patients cured of cancer in childhood visit a dentist’s room

annually (M.W. Yeazel, 2004.).

Aim. Assessment of/to assess the condition of dental health in patients with acute lymphoblastic leukaemia (ALL) in prolonged remission.

Materials and methods. In 2014–2015 a preventive examination in 122 patients cured of ALL who were in remission for 2 years and for 2 and more years was performed at the

Rehabilitation Centre “Russian Field”. A comparison group comprised of 204 children with general somatic pathology who also received a course of rehabilitation at the centre during

this period. Median age of the patients from the primary/initial/treatment group was 7.9 (4–16) years at the moment of examination. Median age of the children from a control

group was 8.5 (4–17) years.

Results. Approximately a half of the patients from the control group (41.3 %) visited a dentist’s room annually. Only 32 % of the patients from the primary/initial/treatment group

visited a dentist every year and usually in order to perform sanitation. During the preventive examination by a dentist from the Rehabilitation Centre “Russkoe Pole” children with

leukaemia show signifi cant changes in hard tooth tissues of carious and noncarious origin both during temporary/transient mixed and during permanent occlusion. The connection

between the severity of pathology and age is detected: the younger a child at the moment of treatment is, the more apparent changes of stomatological status it has.

Symptoms of enamel dysmorphology in the form of demineralization foci, incisor sulci and pits, local hypoplasia, tooth erosion, rapid/acute carious injuries, pulpitis, pulpal polyps,

periodontitis, sometimes increased tooth abrasion are detected in the patients from the primary/initial/treatment group (76 %) considerably more often than in the control group

patients. There are either carious cavities or a plumbic ball in most molar teeth. Simplifi ed OHI-S index was > 1.6 that corresponds to the unsatisfactory and poor oral hygiene in 2/3

of the primary/initial/treatment group patients.

Conclusion. Diffi culty in the management of children with hemablastosis occurs when children are not only afraid of stomatological intervention but also tolerate hygienic

manipulations in oral cavity with eff ort. In addition, up to 30 % of parents and patients refer to doctors’ recommendations about limited hygienic procedures although there are vast

opportunities of using sensitive toothbrushes, medical toothpastes, elixirs. The patients of this group require special facilities/remedies/tools and methods of treatment regarding the

primary disease not only in an oncohematological hospital/in-patient facility but also at a dentist’s room.

A B S T R A C T N O . : O P - 3 5 4

Carbohydrate-lipid metabolism disorder in patients

with oncohematological pathology

E.V. Zhukovskaya

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: tumours, metabolic syndrome, children

Introduction. Metabolic syndrome (MetS) development in children who had received treatment for malignant neoplasms is a fact repeatedly reported in literature

(C. Bizzarri et al., 2015; N.M. Abu-Ouf et al., 2015). Genetic determinants of metabolism, character of nutrition, cultural peculiarities can form a certain specifi city of the occurence

and clinical manifestations of this anticancer treatment complication.

Aim. To investigate the spread of metabolic syndrome in reconvalescents of malignant neoplasms.

Materials and methods. The enrolment of 105 patients admitted to the Rehabilitation Centre “Russian Field” was carried out according to the following criteria: a child is to be

in the fi rst remission for 2 or more years; antitumor therapy compulsorily/defi nitely involved polychemotherapy ± irradiation ± surgical treatment. 38 patients had the diagnosis

«lymphoma» (С82,83); 16 patients – «acute lymphoblastic leukaemia» (С91.0); 15 – «nephroblastoma» (С64); 14 – «soft tissue tumors» (С48,49), 14 – «neuroblastoma» (С47);

8 – «bone sarcomas» (С40,41). In order to detect carbohydrate-lipid metabolism disorders an obligatory investigation of patients was performed: family anamnesis, body mass index

(BMI) assessment, common blood count, biochemical testing, glucose tolerance test (GTT), HOMA (The Homeostatic Model Assessment) index calculation, ultrasound investigation

on indications, consultation with an endocrinologist.

No child was diagnosed with diabetes mellitus. Since it was impossible to confi rm the presence of episodes of carbohydrate metabolism disorder and/or development of steroidogenic

diabetes based on the presented medical documents in all patients, this parameter was not taken into account in the course of the treatment.

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Results. A burdened familial history concerning adiposis/obesity and diabetes mellitus was diagnosed in 62 (59 %) patients; BMI > 25 was registered in 59 (55 %), 27 patients of them

(26 %) had BMI > 30, variants of dyslipidaemia were found/noted/registered in 43 patients (41 %), HOMA index > 2,5 – in 32 (30 %). A stable increase in arterial pressure exceeding

age standards was registered only in 6 adolescents older 14 years, the diagnosis “arterial hypertension” (AHT) was not established in them previously. Individual combination of

symptoms of MetS under consideration was the following: insulin resistance in combination with dyslipidaemia was found in 32 (30 %) patients, only 24 % of them had an increased

BMI, 22 % place of deviation in parameters of Ca metabolism, including high level of osteoporosis markers; all 6 adolescents with AHT were included in the group of children with

MetS. Group of patients with MetS comprised 13 children with lymphomas, 9 – with leukemia, 2 – with nephroblastoma, 2 – with neuroblastoma, 2 – with soft tissue tumors,

4 – with bone sarcomas.

Conclusion. In patients with diff erent oncohematological diseases increased body mass was registered in 59 (55 %) cases, of which insulin resistance (the main symptom of MetS) was

found only in 25 (24 %) observations. No dependence on the diagnosis, the intensity of antitumor therapy performed was detected in this group. Taking into account a high frequency

of MetS among late eff ects from the performed treatment, further data acquisition in order to defi ne the intensity of a dose-dependent eff ect of the use of chemotherapeutic agents,

the role of genetic predisposition in its development, gender and age specifi city is necessary. It is probably useful to transfer MetS from the category of late eff ects from cytostatic

treatment and consider its symptoms during nutrition regulation, moments relating to regime at most early at the stages of a child’s treatment in order to carry out prophylaxis of

further development of life-threatening complications (A.E. Kero et al., 2016).

A B S T R A C T N O . : P - 3 9 9

Second tumours in patients cured of malignant neoplasms in childhood

O. Gerasimova1, A.P. Shapochnik2, A.V. Klimushkin2, L.V. Sidorenko2 1Orenburg Regional Clinical Oncology Dispensary, Russia; 2Federal Research Center of Pediatric Hematology,

Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: second tumours, children

Introduction. Patients cured of malignant neoplasms in childhood are at high risk for developing second tumours. The risk of neoplasms in reconvalescents is approximately 10 times

higher than in general population that seems important due to the increasing number of survivors in whom later second tumours are registered.

Second tumour is the leading cause of death in the long-term follow-up period regarding the patients cured of childhood cancer. Generally these are solid tumours, breast cancer,

thyroid tumours, skin tumours and brain malignant tumours. There is a clear connection between the above mentioned tumours and radiation exposure defi ned by the latency period

exceeding 10 years.

Other nosological entities of second tumours such as leukaemia (myeloid forms or myelodysplastic syndrome) are associated with the use of cytostatic therapy are distinguished by

a shorter induction period. Alkylating agents and/or topoisomerase II derivatives are most commonly discussed among the anticancer drugs responsible for the development of

second tumours.

Aim. To investigate medical frequency characteristics and structure of second tumours in patients cured of childhood cancer.

Materials and methods. A retrospective analysis of hospital database of 398 patients cured of malignant neoplasms in childhood from 1995 to 2010 at Orenburg Regional Clinical

Oncology Dispensary was carried out. The duration of remission was 5–20 years.

Results. The diagnosis of second tumour was made in 5 (1.2 %) patients and this rate is lower than the rate reported by other authors (2–4 %). Explanation for this can be patients’

migration outside the region, underestimation of data about the second tumour already in an adult patient. Thyroid cancer (TC) and cancer of the hairy part of the head were detected

in 2 patients with acute lymphoblastic leukaemia (ALL) aged 18 and 22 with the remission period of 7 and 10 years. The patient with a retinoblastoma aged 12 (9 years after the end

of radiation therapy (RT)) and the patient with a central nervous system (CNS) tumour aged 19 (5 years after the end of complex treatment) were also diagnosed with thyroid cancer

(TC). 6 years after the end of treatment a meningioma developed as a second tumour in 1 patient with ALL aged 20. At the present moment all 5 patients are alive after operative

removal of the second tumour.

Conclusion. All patients received total focal dose of 18–50 Gr at stages of primary tumor treatment. The development of thyroid cancer in 3 cases and solid neoplasms in the zone of

primary tumour irradiation in 2 patients confi rms the fact that radiation therapy (RT) is one of the factors of second tumour induction.

Early detection of pathological conditions associated with the eff ects of antitumor treatment, dynamic follow-up, use of preventive treatment strategies allow to reduce the disease

incidence in adult patients cured of childhood cancer.

Recent studies allow to consider genetic predisposition as a risk modifi cator of the second tumour development. Although these conclusions require further verifi cation, they provide

opportunity to form new approaches that can help to develop eff ective strategies for the prophylaxis of late eff ects in patients cured in childhood.

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A B S T R A C T N O . : O - 0 9 0

Keep up your spirit to conquer cancer

Sazkia Gabriellia Zakaria

Indonesia Childhood Cancer Foundation (YOAI)

Key words: cancer, patient

Introduction. My Name is Sazkia Gabriellia Zakaria. I was born in Tangerang, Indonesia on February 14th 1994. I have leukemia in 2007 when I was 13 years old and fi nished my

treatment in 2009. After fi nishing the treatment I joined YOAI (Indonesian Childhood Cancer Foundation) in 2010 and became a member of Cancer Buster Community/CBC (Childhood

Cancer Survivors Community. My hobbies are singing and writing diaries. I created a song for myself based on my experience as a cancer patient.

Aim. As survivor I would like to motivate other cancer patients to have the same spirit as mine. I have so many goals that I want to complete in my life. I want to help all of my friends

who suff er from cancer by giving them support. They need to keep up their spirit, and never give up.

Materials and methods. Writing Diaries: As a patient we get easily bored. So to kill the time, writing is one of the easy options to express yourself. This can be an inspiration to

become a lyric of song or storybook, and I choose to make a song.

Results. With the help of my family I was able to produce my own album. I will donate 40 % of the income for the cancer patients through the Indonesian Childhood Cancer

Foundation (YOAI). And for other children with cancer throughout the world.

Conclusion. Up to present I am still Writing songs in English and Indonesian. I was able to give several copies of my album to foundations at ICCCPO Conference in Hongkong. I wish

my idea will inspire other cancer patients. I would like to make them believe that every cloud has a silver lining. There must be a blessing behind this cancer we suff er.

A B S T R A C T N O . : O - 0 9 1

Indonesian childhood cancer foundation’s succesful programs and initiatives for survivors

Zanty Zanzibar

Indonesian Chilhood Cancer Foundation

Key words: YOAI Survivors

Introduction. Indonesian Childhood Cancer Foundation / Yayasan Onkologi Anak Indonesia (YOAI) is a foundation which was set up by Parents who have children with cancer. Our

concern for children with cancer are high and would very much like to give them a better life and future. We have been through with the survivors since they were diagnosed with cancer.

One of our activities is to support children with cancer until they survive and become independent. Therefore we made several successful Recovery Programs which consist of

Motivating social interactions and adaptations, giving strong self confi dence and leadership character.

Aim. To improve the quality life of Survivors in all aspects.

Materials and methods. 1. Survivor Cancer Camp: Each year we invite from all regions of Indonesia to gather at a special resort place to get a diff erent environment. They are given

skills on: Creativity, Collaboration, Problem Solving, Communications and Management as well as knowledge on how to overcome the Late Eff ect of Medications.

2. Upgrading & Workshops: The programs are made with the support of Doctors, Psychologists, Nutritionists and Motivators Trainers through Group Discussion and Team Building.

CHILDHOOD CANCER INTERNATIONAL (FOUNDATIONS/PARENTS/SURVIVORS)

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Results. After nine years we notice they become stronger in many ways. Some have become entrepreneurs, artists, lawyers and doctors. Many works for various companies such as

Banks, Hospitals and Hypermarkets. Several got married and have children.

Conclusion. It is proved that children with cancer can be cured and that our Recovery Program has been successfully absorbed by our survivors. They fi nished their educations, become

independent and reached their dreams.

A B S T R A C T N O . : O P - 1 3 8

The central venous catheter (CVC) checklist for anaesthesiologists

D.Sh. Bikkulova

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: CVC

Introduction. A safe insertion of all types of central venous catheters (CVC) has always been of vital importance. There are some factors such as the rotation of employees,

the introduction of new types of catheters, a change in preferences of treating physicians when choosing a central venous access device that can aff ect this process. In order to ensure

a patient’s safety and to control the situation, it is important to use control checklists for CVC insertion.

Aim. It is to provide good clinical practice and prevent complications.

Materials and methods. In the FRC PHOI CVC insertion is performed by anesthesiologists. Since 2013 checklists have been completed annually, from February till May. Upon their

completion, the checklists are analyzed and the work of the medical staff is corrected accordingly.

Results. Over the last three years (2013–2015) (146 + 254 + 406 = 806 (100 %)) checklists have been analyzed. The age range of patients with hematological and oncological

diseases is 2 months – 18 years old. The checklists were completed by 7 doctors annually. This work was carried out in accordance with the hospital protocol. Year in, year out the

number of CVC insertion procedures has been increasing, the proportion of catheters types used has been changing. For instance, in 2013 long-term CVCs were inserted into the

subclavian or the internal jugular vein. There is a high frequency of complications entailed by the insertion of long-term CVCs into the subclavian vein. Thus long-term CVCs are

nowadays inserted only into the internal jugular vein, whereas short-term CVCs – only into the subclavian vein. In 2014 some cases of repeated puncturing of the subclavian vein

performed by 3 doctors were registered. The punctures were performed without US guidance. Further analysis showed that the puncture site and the direction of the needle when

puncturing the subclavian vein had been incorrect. A number of training simulations for doctors were conducted in order to change the technique of the procedure. In addition,

US guidance of the subclavian vein was put into practice. In 2015 there were 78 cases of CVC substitution identifi ed in 40 checklists. The reasons for the substitution included

accidental removal of CVC by a patient 22 cases, a substitution of a long-term CVC for a short-term CVC – 17, thrombosis – 13, infection – 11, an incorrect position of the internal

tip of CVC – 6, a surgery performed near the puncture wound – 1 case. Accidental catheter removal was observed in adolescents aged 14–18 years, and as a result а brochure about

CVC for adolescents was compiled. In order to eliminate the causes of catheter-related infection and thrombosis, a recertifi cation of the staff as well as training for newly employed

nurses, etc. were carried out.

Conclusion. A regular analysis of checklists allows one to identify mistakes and errors in work and individual features of the staff and patients as well as helps to improve

the organizational activity of the hospital administration. And we understand that permanent self-analysis of work provide good clinical practice and prevent complications.

A B S T R A C T N O . : P - 4 0 5

Photohromotherapy alternative method for treatment hemangioma in infants

Yu. Kozel, E. Sheiko, A. Shihlyarova, E. Zlatnik

Rostov Scientifi c Research Institute of Oncology, Rostov-on-Don, Russia

Key words: fotohromotherapy, hemangioma, infants

Introduction. Photochromotherapy is a topical treatment method for hemangiomas in children.

Aim. This study aims to analyse the clinical eff ects of noncoherent monochromatic red light emitting diodes on infantile hemangiomas (IH) children from the new-born to one-and-a

half year old for the period of 2004 to 2015.

Materials and methods. We investigated the eff ect of PHT (photohromotherapy) of red spectrum λ = 635 nm with irradiation dose 3•86–3•96 J/cm2 to 2010 children with IH.

Results. The results showed a signifi cant regression was in 100 % of simple IH; in 92 % of mixed ones and 84.5 % of cavernous forms after PHT. Progression of IH was seen only in

1 patient (0.95 %) subjected to tumor destruction. After the fi rst course of PHT, simple and mixed forms of IH regression were registered in half of the patients (49.4 and 50.2 %

respectively), in cases of cavernous IH – only in 15 % of babies. Ultrasound control demonstrated reduction of tumors` size in 46–71 %, decrease of blood fl ow in 11–22 % and

formation of capsule in 40 % of the cases. After the second and the third courses of PHT proportion of the patients with regression of IH continued to increase. Decrease of tumors`

size was found in 98 %, blood fl ow was weak or absent in 96 % of cases, clear borders or capsule were described in 96 % of cases, the absence of the feeding vessel – in 80 cases.

VEGF levels in sera of patients with IH before the treatment were 221.1 ± 12 pg/ml, which was 3.1 times higher than in healthy children (72.6 ± 3.2 pg/ml). After eff ective PHT VEGF

levels in patients` sera became close to normal (68.9 ± 2.0 pg/ml). In cases of ulcerative complications of IH the positive local eff ect of PHT on suppuration and infl ammation was

observed. The clinical PHT practice has been allowed to grain the eff ects of healing the wound just after the fi rst course (10 procedures). After the second and third courses (about

25–30 procedures), the signifi cantly tumor size decrease up to the regress and formation of scar has been observed. Ultrasound control confi rmed the absence of tumor feeding vessel.

Conclusion. The gaining eff ects of healing the wound by improving the reduced exudative and epitelisation phases, the signifi cantly tumor size decrease up to regress has been observed.

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A B S T R A C T N O . : O P - 4 0 7

The greif care programs in Children’s Cancer Association of Japan (CCAJ)

Hiroko Ishibashi, Satomi Onda, Kaori Nonomura, Asako Katayama, Megumi Yokokawa, Akiko Higuchi

Children’s Cancer Association of Japan

Key words: children, cancer

Introduction. CCAJ Children’s Cancer Association of Japan) is a Japanese non-profi t organization founded in October, 1968 by parents who had lost a child due to cancer. Since its

opening, CCAJ has helped children receive proper and accurate diagnosis and treatments equally, and improved quality of life of patients and their families, with earnest hope to

overcome all pediatric cancer. Thanks to the recent medical progresses in cancer treatments in Japan, approximately 80 % of pediatric cancer are now said to be curable. While 2000

children are diagnosed with cancer and begin their treatments every year, we lose 500 other children to cancer at the same time. As our grief care program, we have been engaged in

providing comfortable and safe places to which they can belong.

Aim. Introducing and sharing our achievements in grief care program who lost a child to cancer together with ones from other countries.

Materials and methods. 1. Listen to what parents want to say and provide consultation by social worker. 2. Facilitate group sessions 8–9 times a ear for mourning parents.

3. Host a short-term intensive support group for mothers who lost a child within a year. 4. Hold regional peer support groups for parents nationwide.

5. Provide events and volunteering opportunities for parents who lost a child.

Results. Starting from peer support groups, we now work with social workers to facilitate various support group sessions as our grief care program: short-term intensive sessions to

help parents release suppressed emotions and share feelings, social groups for specifi c groups of parents (parents who lost their only child without a sibling, or fathers only and so on),

open sessions for everyone to join and more. Some of our parents have started recreational activities themselves, such as outdoor camping by fathers, with other parents with similar

backgrounds. They create their own comfortable and safe space in which they do not need to share their pain or set up a specifi c theme for gathering.

Conclusion. These meetings widen the circle of friends and enable peer support activities to be passed down to the next generations to come. These acts by parents drive us to do

what we have been doing for nearly 50 years. Our social workers at CCAJ are committed to provide various occasions to our parents to have chances to meet other parents who went

through similar experiences. We help them to fi nd their own safe haven where they can feel connected in order to prevent them from being emotionally casted out from society and

being even more saddened by it. Major roles in grief care those parents, and social worker is supporter to draw their power by providing their places.

A B S T R A C T N O . : O - 4 0 8

Client – centered psychological assistance to children with cancer and their families

A.S. Petrova

Regional non-governmental organization “Children and Parents Against Cancer”, Saint-Petersburg, Russia

Key words: psycho-oncology, project of client – centered psychological assistance

Introduction. Childhood cancer is a tragedy for the whole family. First of all, it endangers the child’s life. A sudden change in the way of life of a family and its social status is very

stressful. This crisis point is often critical for family relations. However, the psychological climate in the family is the most important factor infl uencing the treatment process of the

child and its result. We implement the project of Psychological assistance to children with cancer and their families in the children’s Department of the Oncology Institute named after

N.N. Petrov.

Aim. It is important to organize an integrated medico-socio-psychological support of families with children with cancer as well as precise methods of dealing with them.

Materials and methods. The uniqueness of the project due to the application of techniques of client – centered therapy for cancer children and their families. The author of the

method is K. Rogers who based it on building a certain kind of relationship between client and therapist, and leads to a systematic personal growth, accepting responsibility, self-

knowledge.

Results. It helps to accelerate the adaptation process in the Hospital, to make crisis support, to remove the primary stress. Children and parents that coming to the treatment get

the consultation of the psychologist. Many of the primary clients become permanent, allowing to accompany the family at every stage of treatment and support in the process of

accepting the diagnosis.

Conclusion. We would like to share our own experience of the process of the integrated support of each child, allowing to consider interests and personality of patients.

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A B S T R A C T N O . : O - 4 0 9

“Forum of Winners” as a mean of rehabilitation of teenagers

and young adults who suff ered serious illness

A.S. Petrova

Regional non-governmental organization “Children and Parents Against Cancer”, Saint-Petersburg, Russia

Key words: “Forum of Winners”, rehabilitation of teenagers and young adults who suff ered serious illness

Introduction. 11–14 June 2015 it was the First International Forum of Winners. That was the platform for meeting of of teenagers and young adults who have had childhood cancer.

Strengthening patients community is an important tool for rehabilitation and to improve the quality of care for sick children.

Aim. The purpose of The Forum was to strengthen the patient community for reabilitation and sharing the experience.

Materials and methods. The Forum program suggested psychological groups, discussions, presentation of regions of participants, lectures of specialists, exchanging of the

experience (for example the report of the colleagues from Serbia, which engaged in the rehabilitation of adolescents and conducted its training for the Winners) and an informal part.

In the forum participated the representatives from diff erent regions. The St. Petersburg community shared their experience of participation in rehabilitation programs. All of them

have participated in diff erent rehabilitation programs: for example camps.

Results. We managed to combine cured people from diff erent regions of our country. This laid the basis for the formation of a community of winners. Participants learned about the

long-term consequences of childhood cancer, to understand that they are not alone in their experience. The forum gave them the opportunity for open communication with each

other and with experts.

Conclusion. The Forum helped to lay the prospect of continued cooperation of people who suff ered serious illness.They’ve became a team with its purpose to help each other and

children who have the same problems now. We’ve got a lot of thanks and have a plan to make The Forum every two years.

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A B S T R A C T N O . : O P - 0 9 2

Safety of cyclophosphamide infusion in the outpatient setting in pediatric patients

with malignancies: a retrospective review

Amal Al Sabahi1, Diana Bushnak2, Viqaruddin Mohammed2, Ibrahim Al Fawaz2, Amani Al Kofi de2,

Mouab Ayas2, Afshan Ashraf Ali2

1Royal University Hospital; 2King Faisal Specialist Hospital & Research Centre

Key words: cyclophosphamide, safety, hydration, side eff ects, short infusion

Introduction. Some complications of cyclophosphamide (CTX) administration may be hemorrhagic cystitis or renal impairment. This is avoided by giving prolonged hydration

and mesna for bladder protection concurrently. Pediatric patients at our institution receive outpatient cyclophosphamide with mesna and short hydration as a standard of care.

We reviewed the incidence of complications using this method of administration.

Aim. 1) Identify the incidence and severity of complications associated with administration of outpatient cyclophosphamide; 2) Correlate the risk factors that may contribute

to the incidence of complications.

Materials and methods. A review of the medical records of patients age 0–14 years diagnosed with Rhabdomyosarcoma or Ewing Sarcoma treated at King Faisal Specialist Hospital

from January 2005 until December 31, 2012 was conducted. Patients who received outpatient cyclophosphamide, dose range from 1200 mg/m2/dose to 2200 mg/m2/dose every 2 to 3

week, 2 doses of mesna, and a 3-hour hydration (125 ml/m2/hour) were included. The incidence of complications such as Grade III/IV myelosuppression, impairment of renal function,

electrolyte imbalance, hematuria, and hemorrhagic cystitis in this group were reviewed.

Results. A total of 87 patients received Cyclophosphamide infusion over the above time period. 36 (41 %) of the patients had Rhabdomyosarcoma and 51 (59 %) Ewing Sarcoma.

There were 55 female (63 %) and 32 male (37 %). The age at diagnosis was categorized in to three sub-groups: Group-I (0 to 3 years), Group-II (3 to 7 years) and Group-III

(7 to 14 years). There were 20 (23 %), 29 (33%), and 38 (44 %) patients in Groups I, II, and III respectively. Complications related to cyclophosphamide infusion were observed in

43 patients (48 %) of which 25 patients had Ewing Sarcoma and 18 had Rhabdomyosarcoma. 38 patients developed hematuria at some time during their therapy (88 %), febrile

neutropenia was observed in 3 patients (7 %), and 2 patients had hemorrhagic cystitis (5 %). None of the patients required hospitalization for the management of the above

mentioned complications. Renal impairment or grade III/IV myelosuppression was not seen in any of the patients. When analyzing the age subgroups, it was observed that 56 % of

the patients who had complications belonged to Group-I and II. Correlation between the dose of cyclophosphamide and the incidence of side eff ects was not found in this review.

Conclusion. In our setting, cyclophosphamide may be given safely in the outpatient setting with the use of a short 3-hour hydration even in small children. There was no incidence

of increased complications requiring hospitalization or compromise in the treatment for this group of patients. This method is more convenient for the patient and the family and also

potentially more cost eff ective.

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A B S T R A C T N O . : P - 1 1 8

Rehabilitation of children with onco-hematological diseases at Kirov Health Resort “Avitec”

O.M. Tselousova1, N.M. Pozdeev1, A.V. Rylov1, G.G. Anufrieva2 1Kirov Scientifi c Research Institute of Hematology and Blood Transfusion, Kirov, Russia; 2LLC Health Resort “Avitec”, Kirov, Russia

Key words: rehabilitation, children, onco-hematological diseases

Introduction. The treatment of onco-hematological pathologies by means of cytoreductive therapy in 70–90 % of cases entails full recovery of patients 0 - 18 years of age. However,

as a result of the disease and its treatment, some physiological, psychological and social functions are often disrupted and lost. Rehabilitation is a complex of therapeutic and

preventive measures aimed at eliminating or compensating functional defects, boosting the organism’s resistance against damaging factors of the environment and correcting

immune defi ciency, all of which enables the patient’s adaptation, in particular, the patient’s social adaptation, to new life conditions. The most famous children;s rehabilitation centre

for onco-hematological patients in the Russian Federation is the health resort “Russkoe Pole” (the Moscow Region). Since 2015, rehabilitation programmes were put into practice at

the health resort “Avitec” (Kirov).

Aim. To assess the effi cacy of rehabilitation programmes for children with onco-hematological pathologies in the setting of Kirov health resort “Avitec”. Materials and methods. In

2015, 29 patients aged 6 to 16 years old (21 patients – with acute lymphoblastic leukemia, 2 – with acute myeloblastic leukemia, 1 of which – in 1 year after allogenic unrelated

bone marrow transplantation; 6 – with various lymphomas) underwent rehabilitation at the health resort “Avitec”. All children had had complete clinical and hematological remission

without any specifi c treatment for 2.5 years at average (1 to 8 years).

The rehabilitation programme included naturotherapy with phytotherapy, speleochamber, hydrotherapy (a pool with mineral water, baths), art therapy, psychotherapy, remedial

excercises, Nordic walking, games in the open air. The duration of the course constituted 21 days.

Results. The indications for rehabilitation therapy were: a chronic pathology of ENT organs, functional disorders of gastrointestinal tract and toxic liver damage in the form

of cholestasis in all patients. The effi cacy of rehabilitation treatment was noted in all children manifesting itself in the normalization of emotional tonus and sleep, a decrease in the

frequency of acute respiratory viral infections, the absence of aggravation of chronic infections of ENT organs over the observation period of 6 months, recovery of peer communication

skills. These changes are persistent.

Conclusion. The opening of a new rehabilitation centre for children with oncohematological diseases located in the familiar climate and within walking distance creates the conditions

for rehabilitation treatment of patients who for various reasons cannot undergo therapy outside the region. The advantage of Kirov health resort “Avitec” is that the physicians from

the Pediatric Department of Hematology and Chemotherapy of Kirov Research Institute of Hematology and Blood Transfusion can perform regular health monitoring of patients.

A B S T R A C T N O . : P - 1 4 2

The effi cacy of frozen-thawed irradiated washed erythrocytes in children with

hematological malignancies

N.N. Starostin, P.E. Trakhtman

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: cryoconservation of RBC, hematological malignancies, RBC transfusion

Introduction. Modern programs of treatment of patients with hematological malignancies require a long-term transfusion support. The issue of the selection of erythrocyte

suspension (ES) for patients with rare blood groups, sensitized patients is particularly acute in autoblood procurement.

Aim. To perform a comparative analysis of effi cacy of frozen-thawed and washed erythrocytes (T&WE) transfusion and normal ES transfusion to children that received the therapy in

hematological malignancies with anemia syndrome.

Materials and methods. A retrospective analysis of patients with hematological malignancies was performed that received hemotransfusional therapy of the normal ES – group A

(30 patients) and T&WE – group В (28 patients). The main criteria for transfusion was a hemoglobin level decrease less than 80 g/l in the absence of an ongoing bleeding. The process

of glycerolization and deglycerolization was carried out with automatic system of cell processing АСР 215 (Haemonetics, USA). Campared groups were similar by mean mass (group

А – 23.7 ± 5 kg, group В – 25.4 ± 4.2 kg), diagnosis (group А: acute myeloblastic leukemia (AML) – 15 patients, juvenile myelomonocytic leukemia (JMML) – 11, acute lymphoblastic

leukemia (ALL) – 4 patients; group В: AML – 14, JMML – 10 and ALL – 4 patients), indications to transfusion (mean hemoglobulin level in group A was 76.6 ± 4.2 gm/dl, in group

В – 74.75 ± 7.05 gm/dl). Recipients of both groups received volume of ES at 10–12 ml per 1 kg of body weight.

Results. ES characteristics: hemoglobulin – 55.8 ± 3.34 g/dose, erythrocytes – 6.16 ± 0.6 × 1012/l, hematocrit – 56 ± 4.8%. T&WE characteristics: hemoglobulin – 46.6 ± 1.5 g/dose,

erythrocytes – 5.43 ± 0.3 × 1012/l, hematocrit – 48.8 ± 1.3%. In the analysis of hemotranfusions effi cacy the increase of erythrocytic indications was observed inside each group,

however the increase of a hemoglobulin level, number of erythrocytes and hematocrit was signifi cantly lower in the group of patients that received T&WE. The increase of indication

in the group A: hemoglobulin – 23.5 ± 12.1 gm/dl, erythrocytes – 0.8 ± 0.4 х 1012/l, hematocrit – 6.52 ± 3.7%; in the group В: hemoglobulin – 12.9 ± 6.5 gm/dl,

erythrocytes – 0.53 ± 0.27 × 1012/l, hematocrit – 3.2 ± 1.6%. Considering initially lower characteristics of T&WE compared to ES it is possible to assess the effi cacy of transfusion

of erythrocyte-containing environments as positive.

In the analysis of ES and T&WE transfusions tolerance in recipients, the general state of the patient, time pattern of systolic and diastolic blood pressure, pulse rate, breathing, the body

temperature were considered. The follow-up results showed that during and after transfusion there was no development of post transfusional complications.

Conclusion. Thus, the performed studies allow stating the suffi cient effi cacy of both erythrocyte-containing environments for correction of anemia in patients with hemoblastosis.

Alongside this, the performed comparative analysis showed that more frozen-thawed erythrocytes or repeated transfusion in comparison with ES may be required to achieve target

hemoglobulin and hematocrit values in recipient.

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A B S T R A C T N O . : P - 1 7 1

Neurological outcomes in children with extracranial solid neoplasms

by the age of 24 months

L.L. Pankratieva, D.A. Praulova, L.V. Sidorenko, N.N. Volodin, A.G. Rumyantsev

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: malignant neoplasms, infants, complications after polychemotherapy, neurological outcomes, Bayley scale

Introduction. In spite of signifi cant achievements in the treatment of malignant neoplasms in children of the fi rst year of life, neurological complications mainly caused

by the intensifi cation of polychemotherapy (PCT) can have a considerable impact on the outcomes.

Aim. To assess neurological outcomes in children with extracranial solid neoplasms, who underwent PCT within the fi rst 6 months of life.

Materials and methods. We have examined 25 children with malignant neoplasms. Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III, 2006) were applied.

The investigation was carried out at the age of 24 months, under standard conditions in the presence of one of the parents. According to the results of the tasks completed by a child

(cognitive developmental scales, speech function scales – subtests of impressive and expressive speech, motor skill scales – subtests of fi ne and gross motor skills) and parents’ survey

(social-emotional development and adaptive behavior scales) a composite score on certain subtests and a total score on each scale with the use of centile tables were estimated.

The comparison group included 10 children of equal age for correct assessment.

Results. Summary score values on fi ve Bayley-III scales in a group of patients with malignant neoplasms turned out to be statistically much lower.

Conclusion. The children with malignant neoplasms who underwent antitumour treatment in the fi rst 6 months of life belong to the risk group of unfavorable neurologic outcomes

that requires early development of individual rehabilitation plan.

A B S T R A C T N O . : O P - 2 0 6

Effi ciency of photodynamic treatment for recurrent solid tumors in children

N.M. Rostovtsev1, V.G. Polyakov2, A.N. Kotlyarov3, S.G. Kovalenko1

1Chelyabinsk Regional Children’s Hospital, Russia; 2Pediatric Oncology and Hematology Research Institute of N.N. Blokhin Russian Cancer Research Centre, Moscow, Russia; 3South Ural State Medical University, Chelyabinsk, Russia

Key words: photodynamic therapy, solid tumors

Introduction. Recurrent solid tumors of childhood is a challenge for pediatric oncology. The search for new methods to improve the results of local therapy still extremely important.

Aim. To assess direct clinical, radiological and multislice CT results of photodynamic treatment (PDT) with photosensitizers of chlorine family in children with solid tumors.

Materials and methods. In paediatric surgical department of Chelyabinsk regional children’s clinical hospital PDT was conducted to 9 patients with recurrent solid tumors

(Wilms’ tumor, neuroblastoma, germ cell tumors), stage III–IV.

Patients with neuroblastoma prevailed – 41.2 %. The mean age of the whole group was 1.8. PDT was intraoperative following after oncotomy. We used laser equipment “Lakhta

Milon” (Russia). Output laser power was 2.5 W. During treatment the laser power density ranged from 0.1 to 0.8 W/sm2. We used diff erent doses of photo energy – from 300 to 400 J/sm2,

and for tumors deep infi ltrating growth – up to 500 J/sm2. Administered photosensitizer amount was calculated per 1 kg of the patient’s body weight – 0.6–0.8 mg/kg. Exposure

duration depended on tumor size and was from 10 to 30 min. For tumor exposure we used the light guide with end-formed microlens. Case follow-up and dressing changes were

outpatient. Number of appointments starting with primary inspection and up to results of assessment of the chosen treatment (3 years later) was from 10 to 15, in average.

Results. Assessment of PDT effi ciency involved the following criteria: full absence of local recurrence of the tumor; absence of visible (according to US fi ndings) and palpable sings of

the tumor growth; local recurrence in 6 months; no eff ect – continued tumor growth within near post-operational period, according to US fi ndings. In this respect, it should be noted

that the local recurrence was regarded as a positive treatment eff ect. Direct results of PDT were assessed during 1 year. For most cases, this period was long enough for recurrent tumor

detection. Absence of recurrent tumor was observed in 7 (77.7 %) patients, recurrence was observed in 2 (22.3 %) patients. Recurrence was detected only in patients with Wilms’

tumor of neuroblastoma. In these cases photodynamic re-treatment was not preformed due to tumor dissemination. Follow-up monitoring of 7 patients for 6 months to 3 years did

not reveal any recurrence.

Conclusion. The study shows high effi ciency of PDT for solid tumors in children at advanced stages. In this respect, 82.4 % children had no tumor recurrence within 3 years. PDT should

be implemented more widely in treatment for residual and recurrent tumors, and also when radio- and/or chemotherapy are impossible.

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A B S T R A C T N O . : O P - 2 3 5

Metronomic chemotherapy with vinblastine, cyclophosphamide, methotrexate

and celecoxib in progressive or relapsed childhood cancers

Burca Aydin, Canan Akyuz, Neslihan Kalkan, Nilgun Kurucu, Bilgehan Yalcin, Ali Varan, Tezer Kutluk

Hacettepe University Cancer Institute

Key words: metronomic chemotherapy, relapsed solid tumors

Introduction. Metronomic chemotherapy (MC) has been increasingly used for patients with relapsed or progressive cancer. It presents the opportunity of reducing side eff ects of

chemotherapy in heavily pre-treated patients, prolonging survival with acceptable tumor control and improving the quality of life. Mostly small patient groups reported with some

response to diff erent MCs. A regimen with vinblastine, cyclophosphamide, methotrexate and celecoxib was reported with objective responses.

Aim. This study was planned to assess the outcome of the four-drug MC regimen in relapsed or refractory tumors in children.

Materials and methods. From January 2014 to January 2016, 10 children whom have no other chemotherapy option due to cumulative drug doses were given MC and outcome was

evaluated retrospectively. The MC regimen was reported by Andre et al before and consisted weekly vinblastine 3 mg/m2, daily oral cyclophosphamide 30 mg/m2 in fi rst three weeks,

methotrexate 10 mg/m2 twice weekly next three weeks, and celecoxib 100 mg to 400 mg twice daily for 7 weeks.

Results. The median age of patients was 8.5 years old ranged between 3.3 and 18. Tumors were neuroblastoma in four patients and osteosarcoma, Ewing’s sarcoma, infl ammatory

myofi broblastic tumor, retinoblastoma and yolk sac tumor, each in one patient. Patients received MC until disease progressed or completely disappeared as in one patient median

3 months (1–12 moths). One patient with osteosarcoma at the 3rd relapse of the disease continued 12 months after tumor resection. Treatment discontinued and she is still

complete remission 10 months off -therapy without disease. Four patients had stable diseases at median 2.2 months (1–12 months) and still on MC. Five patients had progressive

disease at median 3.8 months (1–5.4 months). At last follow-up, 5 patients (50 %) are alive. One patient with chronic renal failure had hypomagnesemia, hypopotasemia and

hypophosphatemia. Other than this no severe toxicity was observed.

Conclusion. The low-dose and continuous chemotherapy of MC strategy shows encouraging results with increased compliance, less side eff ects, prolonged survival and improved

quality of life. The 4-drug metronomic regimen with vinblastine, cyclophosphamide, methotrexate and celecoxib was a well-tolerated schema. With the objective responses and

one complete response, this regimen presents a reliable treatment option for patients who have limited treatment choice. Clinical trials with larger groups will help understanding

the antitumor eff ect and impact on outcome or quality of life of MC in patients.

A B S T R A C T N O . : P P - 2 5 0

Clinical features, predictors and outcome of posterior reversible encephalopathy syndrome

in children with cancer

Subramaniam Ramanathan, Seema Medhi, Shripad Banavali, Gaurav Narula, Girish Chinnaswamy,

Tushar Vora, Maya Prasad, Brijesh Arora

Tata Memorial Hospital, Mumbai, India

Key words: childhood cancer, magnetic resonance imaging (MRI), posterior reversible encephalopathy syndrome (PRES), seizures

Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is a clinico-radiological syndrome characterized by a neurotoxic state with vasogenic edema which can be life

threatening if detected late. Being a rare occurence, it has limited published data in children affl icted with cancer.

Aim. The aim of this retrospective study was to elucidate the clinical profi le, predisposing factors, imaging features and outcome of PRES in children receiving treatment for various

malignancies.

Materials and methods. Retrospective audit of the clinical data and radiological features of patients with cancer and having PRES diagnosed between October 2004 to December

2015 was conducted and analyzed.

Results. Forty four patients (male:female – 3:1) were diagnosed with a median age of 8 years(range, 1–15). Primary diagnosis were acute lymphoblastic leukemia (n = 29), acute

myeloid leukemia(n = 5), non-Hodgkin lymphoma(n = 5), chronic myeloid leukemia (n = 1) and solid tumors (n = 4). Most common presenting symptoms were seizures which

was observed in all children followed by headache in 31 children (70 %), altered sensorium was in 29 children (66 %) and visual disturbances in 24 children (54 %). Most common

sign was hypertension observed in 41 (93 %) patients. All the patients with ALL were in either in latter part of induction (87 %) or delayed reinduction (10 %) and had hypertension

(steroid induced) or abdominal pain/constipation (55 %) when they developed PRES. CT scan was abnormal in 15 (51 %) patients but was diagnostic in only 31 %. MRI was performed

in 41 patients; all showed abnormalities. Classic hyperintense lesions on FLAIR and Diff usion weighed images were noted in parieto-occipital region in 33 patients (75 %). Frontal lobe

lesions in association with other regions were noted in 15 patients (34 %). Atypical fi ndings (leptomeningeal enhancement & hemorrhage) were noted in 8 % patients. Follow-up

MRI/CT was obtained in 16 patients which revealed residual abnormalities in 5 patients. No patient died of PRES but 5 patients died of other complications. Neurological sequelae were

observed in only 2 of the surviving 39 patients; both had considerable delay in PRES diagnosis. There were no cases of recurrent PRES in our cohort.

Conclusion. PRES is an important clinico-radiological syndrome in patients undergoing chemotherapy for hematological malignancies. High index of suspicion, early DW images on

MRI in children with classic tetrad of symptoms and hypertension confi rms early diagnosis of PRES and ensures good long-term outcome. Aggressive management of abdominal pain

and constipation that can cause acute hypertension and PRES may help reduce chances of PRES and its complications.

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A B S T R A C T N O . : P P - 2 7 1

Study of anticoagulant eff ect of LMWH in children with cancer and thrombosis using

Thrombodynamics, aPTT and anti-Xa activity

M. Gracheva, P. Zharkov, E. Seregina, A. Poletaev, A. Pshonkin, F. Ataullakhanov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: cancer, thrombosis, low-weight heparin, haemostasis, thrombodynamics, anti-Xa activity

Introduction. The drugs of choice in treatment of venous thrombotic episodes (VTE) in children are low molecular weight heparins (LMWH). There is no information on the application

of a new global method of haemostasis assessment Thrombodynamics (TD) in anticoagulation therapy monitoring in children with cancer at the moment.

Aim. New laboratory method of hemostatic assessment TD is sensitive to the anticoagulant eff ect of LMWH according to in vitro studies. In this study TD, aPTT and anta-Xa activity

were used to monitor anticoagulation therapy in children with cancer with detected DVT.

Materials and methods. 35 children aged from 5 months to 18 years who were at the stage of active treatment of cancer were enrolled in prospective study. In all patients DVT was

confi rmed / diagnosed by Doppler ultrasound. On the day of thrombosis detection anticoagulant LMWH therapy was administered to all children at initial dose of 100ME / kg every

12 hours. Anti-Xa activity, aPTT and TD were used to assess the coagulation status of patients. Haemostatic system was evaluated before and at the 4th day of anticoagulant therapy

with LMWH. Hypercoagulability in TD was defi ned as the increase in clot growth rate (V) above the normal range, hypocoagulability – when V was reduced below the normal range.

OriginPro 8.0 (Microcal Software, Nordhempton, USA) software was used for all calculations.

Results. On the day of VTE detection hypercoagulability in TD was detected in 24 children (69 %): mean V = 33.8 um/min (normal range in children is 22–29 um/min), APTT was

shortened in 4 children (11 %). On the 4th day of LMWH therapy TD demonstrated a signifi cant shift to the area of anticoagulation: V decreased in 2.7 times (*P = 0.0001, paired t-test),

aPTT elongated in 1,17 times (*P = 0.0001), but the main part of values were within the normal range (25.1–36.5 sec). On LMWH therapy there was a correlation between anti-Xa

activity and the values of 1/V: Spearman corr. coeff . 0.67 (*P = 0.0001), there was no correlation between anti-Xa activity and aPTT.

Conclusion. TD allows to reveal changes in the clot growth rate on LMWH anticoagulant therapy in children with cancer with thrombosis. Changes of these parameters correlate with

the degree of anticoagulation according to anti-Xa activity. APTT does not reveal the eff ect of LMWH on the haemostasis of children with cancer with VTE.

A B S T R A C T N O . : P - 2 9 4

The case of clinical emergency reinfusion of red blood cells in massive blood loss

A. Karelin, D. Litvinov, E. Spiridonova, P. Trakhtman, V. Shchukin

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: emergency reinfusion, red blood cells, massive blood loss

Introduction. Massive blood is referred to as the loss of 25–49 % of total circulating blood volume. At the same time the decrease in the mass of circulating hemoglobin by 50 %

is accompanied by a decrease in oxygen delivery by 27 %.

Aim. The transfusion therapy is provided by supplementation of intravascular fl uid volume, fi lling the defi ciency of coagulation factors and the level of circulatingred red blood

cells (RBCs). In order to reduce the risk of transfusion reactions and complications of the intraoperative reinfusion autoerythrocytes collected from wound were used. The use of

autoerythrocytes helps to restore the RBC, hemoglobin and hematocrit level 1–2 days after the operation.

Materials and methods. The example of successful use of apparatus RBC reinfusion is the following clinical case of the patient O. (age 13, weight 54 kg, height 153 cm)

with a diagnosis of “Juvenile angiofi broma of the nasopharynx and skull base (the 2nd stage; Fisсh), 3rd relapse”. The laboratory and clinical indicators were within normal ranges.

A surgery – transnasal endoscopic removal of juvenile angiofi broma of the nasopharynx and skull base under the control of CT – based navigation system – was performed. In order

to reduce the risk of intraoperative bleeding, endovascular occlusion of the tumor from the pools of the maxillary right and left facial artery was performed with hydrogel cylinders

(500 μm) a day before the surgery.

Results. The device for autoerythrocyte reinfusion CATs was used during the operation. Intraoperative blood loss reached 1350 ml (38 % of total circulating blood volume).

Intraoperative infusion volume amounted to 3044 ml, which included 2000 ml of ringer solution, 350 ml of gelofuzin, 394 ml of autoerythrocytes with a hematocrit of 60 %, 300 ml

of donor RBCs suspension.

Hemodynamic parameters remained stable within the operation.The laboratory data after the surgery showed no signifi cant abnormalities (the hemoglobin level was 118 g/l,

hematocrit was 34 %). The patient was observed in intensive care unit for 12 hours and was discharged home in satisfactory condition 9 days later.

Conclusion. The use of apparatus autoerythrocyte reinfusion allows to reduce the risk of transfusion reactions and complications, reduces the duration of the observation in intensive

care unit, which reduces the total cost of the therapy.

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A B S T R A C T N O . : P - 2 9 7

The results of treatment of intraoperative blood loss in pediatric оncology

V. Shchukin, E. Spiridonova, Yu. Ovsyannikov

Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia

Key words: intraoperative blood loss

Introduction. In Oncology practice, the initial condition of the patients may contribute to an increased risk of bleeding both during surgery and after it.

Aim. In order to perform the in-time therapy it is necessary to focus both on predicted and real blood loss, assessing the volume of blood loss during the surgery.

Materials and methods. The analysis of the eff ectiveness of intraoperative blood loss was performed in 1311 children with cancerous diseases. The volume of blood loss was

determined by visual assessment of the degree of wipes impregnation with blood, the measurement of blood volume collected by aspirator, the evaluation of hemoglobin

and hematocrit level. The decrease of hemoglobin level down to 80 g/l and hematocrit down to 25 % with prolonged bleeding was the indication for the beginning of blood transfusion.

The indication for fresh frozen plasma (FFP) transfusion included the defi ciency of blood coagulation factors and the threatened coagulopathy bleeding. Thrombocytopenias required

medical correction in case of low platelet levels (less than 100 thousand/ml).

Taking into account the fact that hypothermia signifi cantly reduces the functional activity of platelets, which contributes to the hemorrhagic syndrome in all the performed cases,

intraoperative warming of the patient was performed.

Results. Intraoperative blood loss up to 20 %, 20–40 %, 40–100 %, over 100 % of total circulating blood volume was diagnosed in 84.2 % (1104 children), 13.2 % (173 children),

2.3 % (30 children), 0.3 % (4 children), respectively. The replenishment of blood loss was performed by step method according to the Protocol, which included crystalloids (for patients

with blood loss up to 20 %), infusion therapy and the use of fresh frozen plasma (for patients with blood loss of 20–40 % and activated partial thromboplastin time or protrombin time

increase by 1.5 times), crystalloid/colloids, fresh-frozen plasma and RBC mass in the ratio of 1:1:1 ( for patients with blood loss of 40–100 %). In case of blood loss over 100 % fresh

frozen plasma, red blood cells, platelets in the ratio of 1:1:1 were used. Positive treatment outcomes were achieve in the vast majority of cases (1310 children, 99.9 %). Intraoperative

blood loss was the cause of death of 1 baby due to disseminated intravascular coagulation syndrome.

Conclusion. The therapy of acute intraoperative blood loss in pediatric oncology should be primarily aimed at maintaining of the circulating fl uid volume and cardiac output. The

restoring of the defi ciency of blood corpuscles is to be started at hemoglobin level below 80 g/l.

A B S T R A C T N O . : O - 3 9 0

The remedial treatment of children with malignant solid tumours

at an early postoperative stage

A.V. Petrichenko, E.A. Bukreeva, A.A. Katyzhenkov, K.F. Savlaev, N.M. Ivanova

Research and Practice Centre for Specialized Medical Care for Children named after V.F. Voyno-Yasenetsky, Moscow, Russia

Key words: rehabilitation, solid tumours

Introduction. The problem of the development of rehabilitation programmes for treatment of children after surgeries on malignant tumours is becoming ever more acute due to an

increased amount and a quality growth of high-tech surgeries, the conduct of organ-preserving therapies including those using various implants; as well as due to the use of complex

innovative equipment.

Aim. A reduction of time gaps between stages of local tumour control and adjuvant treatment, a recovery of a maximal quality of life in terms of residual capacity.

Materials and methods. In 2013–2015, rehabilitation treatment at an early postoperative stage was received by 460 patients who had undergone 497 surgeries. The age of the

postoperative patients ranged between 3 months and 18 years with the predominance of school-aged children (72.8 %) and the median age comprising 6.3 years. Surgeries with

the use of waterjet and plasma technologies as well as thermal ablation of tumours had been conducted. Hip and knee endoprosthetic replacements as well as total endoprosthetic

replacements had been carried out. Polychemotherapy was started on day 5–7 after the removal of a tumour in order to prevent local recurrences and distant metastases. On day

1–3 after a surgery, remedial treatment was conducted at intensive care unit, whereas on days 4–21 it was continued in hospital wards. The employed methodologies included

kinesiotherapy, laser therapy, aero phytotherapy, orthopedic correction, orthotic therapy and corset therapy.

Results. Postoperative complications included: healing by second intention in 2 patients, diaphragmatic cupula paresis in 1 child. An improvement in the overall state of health and

in the functions of vital organs was registered in all patients. Patients who had undergone endoprosthetic replacement demonstrated a recovery of motor skills without contractions.

Adjuvant antitumour treatment was carried out for all patients on day 5–7 after a surgery.

Conclusion. Timely multimodal rehabilitation treatment at an early postoperative stage enables a correction of the consequences of special treatment at early stages, which

signifi cantly reduces children’s incapacitation as well as improves their social adaptation and quality of life.

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A B S T R A C T N O . : O P - 4 0 0

Assessment of patient awareness regarding chemotherapy and related supportive

medications in pediatric cancer patients in pediatric oncology hospital

Sarah Mohamed Abdelsamie, Mohamed Abdelrahman, Karim Alfarsi, Nada Mohamed

Childrens Cancer Hospital 57357, Egypt

Key words: caregivers chemotherapy, adverse drug events

Introduction. Patient/ patient caregivers’ awareness of chemotherapy basic information and compliance to preventive measures are important components of cancer care.

Unfortunately, this aspect of care may be overlooked until problems arise. This leads to needless distress and discomfort and in some cases serious clinical consequences. Assessment

of patient/ patient caregivers awareness regarding chemotherapy treatment and related adverse events was run using open and closed questions survey during waiting to receive

chemotherapy cycles.

Aim. To screen pediatric cancer patients /parents awareness regarding chemotherapy medications, supportive medications and related procedures.

Materials and methods. 66 patients/ parents were interviewed to answer 23 questions (total 1518 questions) regarding chemotherapy medications and supportive medications.

Questions were categorized as: Chemotherapy basic knowledge (7 Questions), drug indication (5 Questions), drug monitoring (2 Questions), patient compliance (2 Questions), Local

anesthesia cream usage (3 Questions) and hygiene behaviors (4 Questions).

Results. Regarding Chemotherapy basic knowledge (7 questions), a total of 462 answers by 66 patients were provided (n = 264) were correct while 43 % (n = 198) were incorrect.

Regarding drug indication (5 Questions), a total of 330 answers by 66 patients were provided. 47 % (n = 154) were correct while 53 % (n = 176) were incorrect. Regarding drug

monitoring (2 Questions), a total of 132 answers by 66 patients were provided. 46 % (n = 61) were correct while 54 % (n = 71) were incorrect. Regarding patient compliance

(2 Questions), a total of 132 answers by 66 patients were provided. 73 % (n = 96) were correct while 27 % (n = 36) were incorrect. Regarding local anesthesia cream usage

(3 Questions), a total of negative 198 answers by 66 patients were provided. where all patients were uneducated about the usage of local anesthesia cream usage before needle

insertion. Regarding hygiene behaviors (4 Questions), a total of 264 answers by 66 patients were provided. 38 % (n = 100) were correct while 62 % (n = 164) were incorrect. From

66 patients 32 were receiving corticosteroids. 44 % (n = 14) were aware of corticosteroid role in chemotherapy regimen while 56 % (n = 18) were unaware of it. From 66 patients

53 were on Trimethoprim prophylaxis during chemotherapy while 13 were not on Trimethoprim prophylaxis due to medical reasons as advised by the physician. From the 53 patients

on Prophylaxis, 47 % (n = 25) were aware of Trimethoprim role during chemotherapy while 53 % (n = 28) were unaware of it.

Conclusion. Improvement plans were needed to enhance Patient/ patient caregiver awareness regarding chemotherapy basic information and supportive medications.

A B S T R A C T N O . : O P - 4 0 1

Assessment of patient awareness regarding chemotherapy side eff ects in pediatric cancer

patients at a pediatric cancer hospital

Sarah Mohamed Abdelsamie, Mohamed Abdelrahman, Karim Alfarsi, Nada Mohamed

Childrens Cancer Hospital 57357, Egypt

Key words: chemotherapy adverse, events, patient, awareness

Introduction. Chemotherapy side eff ects have a profound eff ect on the person with cancer, causing discomfort, longer hospital stays, and in some situations death. Patient/ caregivers

awareness of common side eff ects is an important aspect of cancer care. The aim of the study was to assess patient knowledge and dealing with common chemotherapy side eff ects.

Aim. To screen pediatric cancer patients /parents awareness regarding dealing with chemotherapy side eff ects.

Materials and methods. 66 patients/ parents were interviewed to answer 24 questions (total 1584 questions) regarding chemotherapy side eff ects. Questions were categorized as:

Constipation (3 Questions), Diarrhea (3 Questions), Emesis (3 Questions), Hair Loss(3 Questions), Oral Mucositis (3 Questions), Nausea & vomiting (3 Questions), Fever (3 Questions),

Tiredness (3 Questions. From total 1584 questions, 1152 questions were inapplicable on patients who did not experience some chemotherapy side eff ects while 432 questions were

analyzed).

Results. 1152 questions were inapplicable on patients who did not experience some chemotherapy side eff ects, while 432 questions were applicable. From 432 answers, 58 %

(n = 250) were correct while 42 % (n = 182) were incorrect. Of 66 patients 30 % (n = 20) suff ered from Fever while 70 % (n = 46) did not experience Fever, with appropriate awareness

of cause 80 % (n = 16), dealing with fever 100 % (n = 20), prevention 65 % (n = 13). Of 66 patients 27 % (n = 18) suff ered from mucositis while 73 % (n = 48) did not experience

mucositis with appropriate awareness of cause 83 % (n = 18), dealing with mucositis 39 % (n = 7), prevention 28 % (n = 5). Of 66 patients 38 % (n = 25) suff ered from Emesis while

62 % (n = 41) did not experience emesis, with appropriate awareness of cause 80 % (n = 20), dealing with emesis 76 % (n = 19), prevention 32 % (n = 8). Of 66 patients 23 %

(n = 15) suff ered from nausea while 77 % (n = 51) did not experience nausea, with appropriate awareness of cause 87 % (n = 13), dealing with nausea 73 % (n = 11), prevention 27 %

(n = 4). Of 66 patients 18 % (n = 12) suff ered from Constipation while 82 % (n = 54) did not experience Constipation, with appropriate awareness of cause 50 % (n = 6), dealing with

Constipation 33 % (n = 4), prevention 25 % (n = 3). Of 66 patients 21 % (n = 14) suff ered from Diarrhea while 79 % (n = 52) did not experience Diarrhea, with appropriate awareness

of cause 79 % (n = 11), dealing with Diarrhea 50 % (n = 7), prevention 21 % (n = 3). Of 66 patients 33 % (n = 22) suff ered from Hair Loss, while 67 % (n = 44) did not experience Hair

Loss, with appropriate awareness of cause 91 % (n = 20), dealing with hair loss 64 % (n = 14), prevention 45 % (n = 10). Of 66 patients 21 % (n = 18) suff ered from Tiredness while

79 % (n = 52) did not experience Tiredness, with appropriate awareness of cause 67 % (n = 12), dealing with tiredness 61 % (n = 11), prevention 22 % (n = 4).

Conclusion. Patient/ patient caregivers awareness regarding chemotherapy side eff ects was unsatisfying, which required more eff orts towards improvement.

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A B S T R A C T N O . : P - 4 0 6

Modern methods of venous access in pediatric oncology

Yu.Yu. Kozel, N.A. Maksimova, E.Yu. Semiletkina, S.A. Kuznetsov, G.A. Mkrtchyan, M.V. Starzeckaya

Rostov Research Oncology Institute, Rostov-on-Don, Russia

Key words: central venous catheter, peripheral access, pediatric oncology

Introduction. Treatment of oncological diseases requires prolonged usage of chemotherapy drugs. Unstoppable treatment and laboratory control is available in case of presence of

permanent venous access.

Aim. Choose of optimal way of venous access (VA) for treatment of children with oncological diseases. Modern methods of treatment and arsenal of drugs for pediatric oncology

requires long-term, sustainable, safe and applicable VA.

Materials and methods. Retrospective analysis of diff erent variants VA for 532 patients from 1 month to 17 y.o. treated in FSBI RROI was performed. Patients were divided on

3 groups. Four hundred seventy three patients included to the fi rst group received catheter for subclavian and femur veins. Second group – 21 children with port catheter and third

(38 patients) which were treated with central venous (CV) catheter implanted thru peripheral access (PICC). The analysis of frequency and types of complications after the usage of

above mentioned catheters with diff erent types of VA was done.

Results. Complications associated with implantation and usage of external central venous catheters (CVC) were registered in 32.9 % of cases. Complications in case of port-catheters – in

66.5 % of cases. The majority of problems were presented as infection problems required antibacterial treatment, second surgery and urgent removing of port-catheter. From 21

implanter port-catheters, 14 were urgently removed.

From 2013 we installed 38 peripheral implanting catheters PICC type. Rate of complications with this type of catheters were 18.2 %. One catheter was removed due to suspicion

on infection. Catheter continuity violation was registered at 2 patients. Three patients removed catheters by themselves. Hematoma of lower third part of shoulder was revealed

at 1 patient due to initial thrombocytopenia. Violation of catheter crossability due to it’s thrombosis was not observed.

Thus, the data of the investigations shows that usage of subclavian venous access (SVA) for CVC implantation in comparison with CV and ports have the several advantages:

a. SVA implantation is safe and small-invasive;

b. Terms of SVA catheters usage from 6 days to 1 year;

c. Presence of Groshong vessel makes catheter safe for bleeding and air embolism;

d. Thrombocitopenia and hyperplasia of lymph nodes is not absolute contraindication for SVA catheter implantation

e. SVA catheter implantation do not provides any troubles for patients;

f. In cases of needed SVA catheter can be removed in any medical institution or at home;

g. SVA catheter usage is painless;

h. There is no damage of SVA catheters;

i. SVA catheters implantation can promote the decreasing of frequency of infection complications because the bacterial load on sq.m. in the area of shoulder is less than neck and chest.

Conclusion. From all methods of VA, included in this work, the peripheral, used for PICC-catheters implantation is less invasive, safe, sustainable, long-term, easy in usage. This type

of VA is the method of choice in pediatric oncology.

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CURRICULUM VITAE

Bui Chi Bao, PhD

Principle Investigator of Neuroscience Lab

The Center for Molecular Biomedicine, University of Medicine and Pharmacy Hochiminh city

217 Hong Bang, District 5, Hochiminh city, Vietnam

Email: bcbao@ump.edu.vn

I was training for the genomics and proteomics at Samsung Biomedical Institution (Seoul, Korea) 2007-2012 and recieved my

PhD in Sungkyunkwan University, School of medicine. My pivotal study elucidated genomic instability in children cancer. In 2013,

I subsequently trained for structural biology to screen novel druggable targeted membrane protein at Pediatric Oncology Branch,

National Cancer Institute, US. In 2014, I joined the visiting staff of Blizard Institute, Bart and the London, School of Medicine to

study the cancer epigenetic model in vitro and in vivo. Back to home, I am working at the Center for Molecular Biomedicine,

University of Medicine and Pharmacy Hochiminh, which studies and establishes the Toward Personalized Cancer Medicine Program

to help inform cancer treatment decisions for children patients. My lab focuses on the way that specifi c proteins cause disease

particular in the epigenetic driver for tumorigenesis. My lab has also highly involved in the development of molecular diagnostics

for MYCN, TrKA, TrKB, and ALK mutants in Neuroblastoma cancer indicative of druggable effi cacy. Combining with the clinical

assessments (Imaging, Histology, Pathology, and Surgery) and the biological presentations, his current main goals of biomedical

research are tapping into: 1) Development of the high throughput technologies to document novel biomarkers that are important

to determine the patient outcomes; 2) Understanding the comprehensive cancer profi ling and describing the mechanisms that

lead to diseases; and 3) Characterising novel drugs and targets for cancer therapy. In this SIOP-ASIA conference, I will outlines

the heterogeneity of Neuroblastoma study in Vietnam, the limitations of current methods of copy number variations, mutation

detections and shares data demonstrating the novel clinicopathological signifi cance of COL11A1 in Neuroblastoma progression.

WINNERS OF SIOP ASIA – 2016 CONGRESS SCHOLARSHIPS

182

CURRICULUM VITAE

r. Atish Narayanrao Bakane

Room no. M, Doctors Hostel, Southern Railway HQ Hospital, Ayanavaram, Chennai, Tamil Nadu, India,

PIN 600023.

atishalways2000@gmail.com ( +919884370549)

Registration number: 2006082968

Professional Qualifi cations:

1. MBBS – Govt Medical College and Hospital, Nagpur, Maharashtra, India.

2. DCH (Diploma in Child Health) – Seth G.S. Medical College and King Edward Memorial hospital, Mumbai, Maharashtra, India.

3. DNB (Diplomate Of Natinal Board in Paediatrics) – Southern Railway Headquarters Hospital, Chennai, TN, India.

4. FNB (Fellowship Of National Board in Pediatric Hemato-oncology ) – Sir Gangaram Hospital, New Delhi, India.

Work Experience:

1) Worked as Medical Offi cer and Consultant Pediatrician for govt of Maharashtra, India.

2) Worked as Consultant Pediatrician in private corporate hospitals in Mumbai, Maharashtra, India.

3) Worked as Registar in Department of Pediatric Hemato Oncology and Stem cell transplant unit.

4) Working as Fellow in Department of Pediatric Hemato Oncology and Stem cell transplant unit.

Professional Affi liations:

Member, Indian Academy Of Pediatrics (IAP), India

Member, PHO (Pediatric Hemato-Oncology) Chapter, India

Presentations:

1. Oral paper presentation in PHOCON (Pediatric Hemato-Oncology Conference, 2015).

Topic: Correlation of CMV reactivation post haematopoietic stem cell transplant with type of graft and its impact on viral

monitoring.

2. Poster presentation PHOCON (Pediatric Hemato-Oncology Conference, 2015).

Topic: Low cost innovations in supportive care in children undergoing high dose chemotherapy.

Publications:

1. Revathi Raj, MRCPATH, Ramya Uppuluri Jr., MD, Divya Subburaj Jr., MD, Sreejith Ramachandran Jr., MD, Atish Bakane Jr.,

MD,V. Lakshmanan Sr., MD. A Single Centre Experience on the Use of DONOR Lymphocyte Infusion in Children Transplanted for

Benign Disorders. Biology of Blood and Transplantation, March 2016, Volume 22, Issue 3, Supplement, Page S145.

2. Revathi Raj, MRCPATH , Sreejith Ramachandran Jr., MD, Ramya Uppuluri Jr., MD , Divya Subburaj Jr, MD, Atish Bakane

Jr., MD, V. Mythili Sr., MD,M. Venkatadesikalu Sr., MD. A Single Centre Study from India on the IMPACT on Quality of Life in

Thalassaemia Major – HSCT Versus LONG TERM Transfusion. Biology of Blood and Transplantation, March 2016, Volume

22, Issue 3, Supplement, Page S192.

183

CURRICULUM VITAE

Name: DR SAADIA ANWAR

Husband’s Name: Muhammad Basharat Bashir

Date of Birth: December 01, 1975

Sex: Female

Mailing Address: 425, Block III, Sector C-1 , Township Lahore, Pakistan

E-mail: drsdnwr9@gmail.com

Domicile: Lahore (Punjab)

Nationality: Pakistani

N.I.C No: 35202-7459892-8

PMDC No: 36748-P.

Mobile No: 0092-322-4228500

Tel No: 00-92-42-35121425

PROFESSIONAL & ACADEMIC QUALIFICATIONS:

ACADEMICS:

– F.C.P.S 2009 Pediatric Medicine (Fellow of College of Physicians & Surgeons Pakistan )

– M.B.B.S. 2000 Fatima Jinnah Medical College For Women, Lahore, Pakistan.

– F.Sc 1994 Lahore College For Women, Lahore, Pakistan.

– Matriculation 1991 Government Madrassa Tul Banat High School 15-Lake Road, Lahore Pakistan.

PROFESSIONAL EXPERIENCE

As Supervisor in Paediatric Haematology &Oncology(Post FCPS, Second fellowship)

– From December 26, 2014 till to date in Paediatric Haematology &Oncology.

– The Children Hospital & Institute of Child Health, Lahore Pakistan

As Assistant Professor Pediatric Haematology &Oncology:

– From September 09, 2011till to date in Paediatric haematology &Oncology.

– The Children Hospital & Institute of Child Health, Lahore Pakistan.

As Senior Registrar Pediatric Hamatology & Oncology:

– Two years in Pediatric Haematology&Oncology (01/09/2009 – 08/09/2011)

The Children Hospital & Institute of Child Health, Lahore Pakistan.

As Resident Medical Offi cer:

– TSeven Years (01-11-2002 to 31-08-2009)

The Children’s Hospital & Institute of Child Health, Lahore

The Children’s Hospital & ICH is a 680 bedded tertiary care teaching hospital, providing all kinds of services related to Pediatrics under one roof. Pediatrics Department including

neonatology, intensive care, cardiology, neurology, nephrology,gastroenterology, infectious diseases, endocrinology, emergency, oncology & hematology, developmental pediatrics,

& general pediatrics . This hospital is a state of the art, referral centre for cancer patients in Pakistan and more than 700 new patients are being registered each year.The Department

of Hematology and oncology constitutes 60 bedded indoor unit, with a four bedded ICU, three bedded Palliative Care unit and a separate Onco pharmacy. The unit also has well

established Out Patient Department with a Follow Up clinic, Thalassemia Clinic,& chemotherapy bay.

Supervisor workshops

– “Assessment of competence”16-19 December, 2013

– “Educational Planning Evaluation “ 01-04 January, 2014

– “Supervisory Skills” 20-23 May, 2014

– “Research Methodology “09-12 December, 2014

– Basic Life support course (BLS),4th July, 2015

– Paediaric Advance Life support course 15-16th January, 2016

RESEARCH WORK

PUBLICATIONS

– Dissertation on “Spectrum of clinical presentation of Juvenile Rheumatoid Arthritis at The Children Hospital” in The Children’s Hospital & Institute of Child Health, Lahore.

– Case Report “Hyper IgE Syndrome with Burkitts Lymphoma Rarer Presentation of a Rare Disease”, Pak Pediatric J 2013;37(2):126-29.

– Abstract publication in international Journal of Blood ,in 2013 “Experience of Non Hodgkin Lymphoma at the Children Hospital & ICH Lahore”

– Case Report “Primary Renal Ewing,s Sarcoma: A Rare Entity “ Journal of the college of Physicians and Surgeons Pakistan 2014,vol.24(special supplement 1 ):S66-S67.

– Two Abstracts published in International Journal of Blood ,in 2014

i) Demographics and disease response evaluation in pediatric high risk acute lymphoblastic leukemia (ALL) patients at a tertiary care centre

ii) “In patient induction mortality in childhood acute lymphoblastic leukaemia; can we combat infection? experience at a tertiary care centre”.

INTERNATIONAL FREE PAPERS

– 45th Cogress of the International Society of Pediatric Oncology September 2013,(SIOP) held at Hong Kong ,Poster presentation “Experience of Non Hodgkin

Lymphoma at the Children Hospital & ICH Lahore”.

– Two poster presentations in 46th Congress SIOP (the International Society of Pediatric Oncology ) 2014 named

i) Demographics and disease response evaluation in pediatric high risk acute lymphoblastic leukemia (ALL) patients at a tertiary care centre

ii) “In patient induction mortality in childhood acute lymphoblastic leukaemia;

184

CURRICULUM VITAE

Name : Dr. Smitha H. V

Date of birth : 26 Aug 1985

Address : Dr. Smitha H.V, Institute of Child Health, Sir Ganga Ram Hospital

New Delhi - 110060, India

Contact : Mob: (+1) 2407762833

Email : drsmithavasu@gmail.com

Education

MBBS, May 2003- March 2009, Jagadguru Shree Shivarathreeshwara Medical College, Mysore, Karnataka, India Rajiv Gandhi

University of Health Sciences (RGUHS), Karnataka, India

MD (Pediatrics), May 2010 – Apr 2013, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India Rajiv Gandhi University

of Health Sciences(RGUHS), Karnataka, India

FNB(Pediatric Hematology-Oncology), Mar 2014 –To date, Sir Ganga Ram Hospital (SGRH), New Delhi, India National Board of

Examinations (NBE), New Delhi, India

Work experience

Senior Registrar in department of Pedaitrics, May 2013 – Sep 2013, Narayana Hrudayalaya Group of Hospitals, Mysore, Karnataka, India

Registrar in NICU, Oct 2013 – Feb 2014, Cradle Hospital, Koramangala, Bangalore, Karnataka, India

Professional Licensure/ Registration

Karnataka Medical Council: 83586,

Delhi Medical Council: 67303

Clinical experience :

Fellow in Pediatric Hematology Oncology

National Board of Examinations (NBE) Trainee-Fellow in Pediatric Hematology-Oncology (PHO) at the Division of Pediatric

Hematology-Oncology and Bone Marrow Transplant Unit, Institute of Child Health at Sir Ganga Ram Hospital (SGRH), New Delhi,

India.

185

CURRICULUM VITAE

NAME- Dr Prateek Bhatia;

M.D. (Path) D.N.B, C.Cy, P.G.D.H.M. MNAMS MIPHA FRCP (Part-1 Hemat)

PRESENT TITLE AND AFFILIATION

Assistant Professor, Pediatric Hematology-Oncology Unit (Hematology-Lab), Department of Pediatrics,

Advanced Pediatric Center, Postgraduate Institute of Medical Education & Research, Chandigarh 160

012, India. http://www.pgimer.nic.in/

DATE OF BIRTH 16 October 1978

NATIONALITY Indian

Education

Degree-Granting Education

Medical School: Government Medical College, Amritsar; M.B.B.S, 1996-2001.

Post-Graduate Training

Residency, Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, 2002-2005.

CREDENTIALS

M.D., Pathology, India, 2005

D.N.B., Diplomate, National Board of Examinations, India, 2005

P.G.D.H.M., Post Graduate Diploma Hospital Management, India, 2006

CCy Certifi ed Cytometrist, International cytometry certifi cation (ICC), 2014

FRCP (Hematology-Part 1)

REGISTRATION AND LICENSURE

Punjab Medical Council, MBBS: 32588, dated 28.01.2002

MD: No 32588, dated 22.09.2008

E-MAIL

prateekbhatia@rediff mail.com/ prateekbhatia16@gmail.com

PHONE

9417186867 / 9914208329 (Mobile). 91-172-2755329 (Offi ce)

EMPLOYMENT HISTORY

Assistant Professor, Pediatrics (Regular). Advanced Pediatric Center, PGIMER, Chandigarh, India, 15 Oct 201, Till Date, In-

charge of Hematology laboratory functioning

Assistant Professor, Pediatrics (Adhoc). Advanced Pediatric Center, PGIMER, Chandigarh, India, 19 Sept 2011, 14 Oct 2015,

In-charge of Hematology laboratory functioning

Senior Research Associate.

Department of Hematology, PGIMER, Chandigarh, 12 May 2010, 18 Sep 2011, Investigator for the project: Detection of few

common chimeric fusion transcripts in ALL cases using the Reverse Transcriptase Polymerase chain reaction assay. During SRA

worked in the Molecular Hematology laboratory, Department of hematology, PGIMER, Chandigarh. Standardized the RT-PCR

technique for detection of common chimeric fusion transcripts in ALL and AML cases. Also worked upon standardization of RQ-

PCR for detection of MRD in CML cases. Trained hands on in all important molecular methods from RNA-DNA extraction and

electrophoresis including putting up PCR’s. Also used to put up immunofl uorescent staining in AML-M3 cases.

Assistant Consultant, Blood Transfusion services, Delhi NCR , 12 Mar 2010, 11 May 2010, Blood banking reporting with

antibody identifi cation, aphaeresis, donation camp organization, education of staff and other related activities

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Senior Resident, Hospital Administration

Department of Hospital Administration, PGIMER, Chandigarh, 1 March 2009 ,11 Mar 2010, Gained administrative experience in

all the support services of The Hospital and worked as Resident In charge of Emergency services, Nehru Hospital. Also worked

in the capability of Resident In charge of Advanced Cardiac Centre, PGIMER, 200-bedded advanced centre of excellence. Gained

experience in convening institute meetings, writing minutes, notices, circulars and Offi ce orders. Worked actively in framing of

the Hospital Infection Control manual and the Disaster control manual.

Consultant Pathologist, Indus and Ivy Pathology Lab, Mohali, Punjab ,1Dec 2008, 20 Jan 2009, Reporting of CBC,

Histopathology, FNAC, Biochemistry and Bone marrow smears and looking after blood transfusion and blood banking services

Senior Resident, Pathology, PGIMER, Chandigarh, 1 Aug 2005, 31 July 2008, Had 12 months posting in Hematopathology

(including blood banking); 12 months Surgical Histopathology, including 6 months Autopsy; 6 months each in Immunopathology

and Cytopathology

Junior Resident, PGIMER, Chandigarh, 1 July 2002, 30 June 2005, During my degree period, I completed 11 months posting

in Hematopathology, 9 months posting in Histopathology, 6 months posting in Cytology, 5 months in Autopsy, 5 months in

Immunopathology. Performed around 150 Adult & Pediatric autopsies; Presented Seminars, CPC’s, Journals. Successfully

completed a research project (thesis) entitled: Study of incidence of CMV infection in pre-term infants/neonates by pp65

Antigenemia Assay, under the able guidance of Additional Prof Dr. R.W.Minz, Head Dept. of Immunopathology

THE NATIONAL SOCIETY OF PEDIATRIC HEMATOLOGISTS AND ONCOLOGISTS

ABSTRACT BOOK

NSPHO

May 25–28, 2016

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