SCIENTIFIC LETTER

An Infant with Primary Autoimmune Neutropenia andInfection by Chlamydia pneumonia : An Unusual Association

Vanessa Laveglia & Laura Garriga-Grimau

Received: 7 May 2013 /Accepted: 29 August 2013# Dr. K C Chaudhuri Foundation 2013

To the Editor: We report a 3-mo-old girl child admitted withfever, rhinorrhea and irritability. The nasopharyngeal aspiratesample confirmed Respiratory Syncytial Virus A infection,and severe neutropenia (340 cells/μL) was observed on theblood test. She remained febrile during the first week, withpersisting neutropenia (260 cells/μL) and a gradual increase ofthe C-reactive protein (CRP) level, up to 82 mg/L. A completeassessment of both humoral and cellular mediated immunityresponse, along with the classical and alternative complementpathways, ruled out a possible associated immunodeficiency.Testing for human immunodeficiency virus, cytomegalovirus,rubella, and toxoplasma antibodies were negative. The patientpersisted to be neutropenic (740 cells/μL) at the time ofdischarge, and a high percentage of anti-neutrophil antibodieswere found to be positive using a combination of immunoflu-orescence and agglutination techniques. Ten days later shewas re-admitted with fever, neutropenia (500 cells/μL) andan increased CRP (272 mg/L). A systemic infection wassuspected, however, chest radiography was clear, and blood,urine and cerebrospinal fluid cultures were sterile. She wastreated with cefotaxime and 5 mcg/kg/d dose of Granulocytecolony-stimulating factor (G-CSF). She remained afebrileafterwards, with gradually decreased inflammatory parame-ters but persisting neutropenia (0 cells/μL). The patient devel-oped diarrhea, a stool sample was obtained, testing positivefor a Rotavirus infection. Seven additional doses of G-CSFwere administered with the last three being increased to10 mcg/kg/d. On the tenth day, auscultation revealed left basal

hypophonesis and chest radiography showed left lower lobeinfiltrate. Chlamydia pneumoniae IgM titres were elevatedconfirming the infection, and treatment with azythromycinwas given (Fig. 1). From this moment the patient had normalabsolute neutrophil count and the treatment with prophylacticantibiotic, co-trimoxazole to prevent bacterial infections wasunnecessary.

Primary autoimmune neutropenia is usually associated withmild infections despite severe neutropenia in children aged 5–15 mo. Around 95 % of cases resolve spontaneously between 7and 30mo [1]. It is characterized by the presence of granulocyte-specific antibodies, however these will only test positive duringthe first detection in 75 % of the cases. Therefore, if there is ahigh index of suspicion then further tests should be carried out,in order to prevent a bone marrow study being required [2, 3].The differential diagnosis includesmainly the cyclic neutropeniaand severe congenital neutropenia. Complicated infections areseen in only 12 % of patients due to the response of a normo orhypercellular with myeloid hyperplasia bone marrow. The first-

Fig. 1 Frontal chest radiograph showing left lower lobe infiltrate

V. Laveglia : L. Garriga-GrimauDepartment of Pediatrics, Hospital del Mar, Parc de Salut Mar,Universitat Autònoma Barcelona, Barcelona, Spain

V. Laveglia (*)Passeig Marítim de la Barceloneta 25-29, 08003 Barcelona, Spaine-mail: 60545@parcdesalutmar.cat

Indian J PediatrDOI 10.1007/s12098-013-1243-0

line treatment in severe infections or prior to elective surgery isthe administration of G-CSF [4].

Lower respiratory tract infections are not commonly relatedto this disease and intracellular bacteria such as Chlamydiapneumoniae are an extremely rare cause of pneumonia ininfants under 6 mo, as in our case.

Acknowledgments Authors would like to appreciate Nuria López-Segura, Consultant Pediatric, Hospital del Mar, Barcelona, Spain, forgiving valuable input on the management of the patient.

Contributions VL, the main author of the article, participated in writ-ing and critical analysis of the text. LGG, participated in literaturereviewing and writing of the text. All of the authors approved themanuscript.

Conflict of Interest None.

Role of Funding Source None.

References

1. Capsoni F, Sarzi-Puttini P, Zanella A. Primary and secondary autoim-mune neutropenia. Arthritis Res Ther. 2005;7:208–14.

2. Sella R, Flomenblit L, Goldstein I, Kaplinsky C. Detection of anti-neutrophil antibodies in autoimmune neutropenia of infancy: A mul-ticenter study. Isr Med Assoc J. 2010;12:91–6.

3. Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course ofautoimmune neutropenia in infancy: Analysis of 240 cases. Blood.1998;91:181–6.

4. James R, Kinsey S. The investigation and management of chronicneutropenia in children. Arch Dis Child. 2006;91:852–8.

Indian J Pediatr