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Incidencia de las enfermedades hematológicas malignas en Europa
por subtipos morfológicos : resultados del proyecto HAEMACARE
Pr Marc MaynadiéRegistre des Hémopathies Malignes de Côte d’Or
EA 4184 – Université de Bourgogne
Proyecto financiado por la Comunidad Europea
Para aumentar la disponibilidad y la estandarización de datos de registros en Europa
Iniciado dentro del programa EUROCARE IV (93 Registros europeos)HAEMACARE : 44 registros - 20 países - 2000-2002 - > 97 000 casos
5 regiones : Norte : países nórdicosInglaterra y Irlanda : UK Centro : Francia, Alemania, Bélgica, Holanda,..South : España, Portugal Italia, Malta,….
Groupos de enfermedades
• Usar la ICD-O-3 para analizar los datos• Hacer grupos de enfermedades para conducir
análisis de sobrevivencia a diferente nivel de detalles
• Grupaje con hierarchia : estrategia usada en el consorcio INTERLYMPH (Morton L et al, Blood, 2007)
Hierarchical group
WHO categories ICD-O-3 codes
ICD-O-2 codes*
1 2 3 4 5 6
Classical Hodgkin lymphoma, lymphocyte-rich 9651
9657, 9658
LN HL HL-CHL-C-LR/MC/LD HL-C-LR
Classical Hodgkin lymphoma, mixed cellularity 9652
9652
LN HL HL-CHL-C-LR/MC/LD HL-C-MC -
Classical Hodgkin lymphoma, lymphocyte-depleted 9653-9655
9653-9655
LN HL HL-CHL-C-LR/MC/LD HL-C-LD -
Classical Hodgkin lymphoma, nodular sclerosis 9663-9667
9663-9667
LN HL HL-C HL-C-NS - -
Classical Hodgkin lymphoma, NOS 9650, 9661, 9662
9650, 9661, 9662
LN HL HL-C - - -
Nodular lymphocyte predominant Hodgkin lymphoma 9659
9659, 9660
LN HL HL-NLP - - -
Morton L et al, Blood, 2007Turner JJ et al, Blood, 2011
Linfoma de Hodgkin
Crude incidence rate/100 000inh/y
by age
by region
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
0.5
1
1.5
2
HL NOS/classical
HL, lymphocyte- rich
HL, lymphocyte depletion
HL, nodular sclerosis
Year of diagnosis
Cru
de
In
cid
en
ce R
ate
s x
10
0,0
00
Année
Ta
ux s
tan
da
rdis
és m
on
de
(é
ch
elle
lo
g)
1980 1985 1990 1995 2000 2005 2010
0.1
0.5
1.0
5.0
10
.05
0.0
Homme
0.1
0.5
1.0
5.0
10
.05
0.0
Année
Ta
ux s
tan
da
rdis
és m
on
de
(é
ch
elle
lo
g)
1980 1985 1990 1995 2000 2005 2010
0.1
0.5
1.0
5.0
10
.05
0.0
Femme
0.1
0.5
1.0
5.0
10
.05
0.0
Incidence estimée HommeIncidence estimée FemmeMortalité estiméeMortalité observée
Scenario= C ==> Figure 3: Tendance chronologique pour la France
Incidence and mortality estimates in France by Hodgkin L
ICD-0-3 code N° de casos Todos Hombres Mujeres
L de las celulas B maduras 42 855 19.14 21.30 17.07
LLC/SLL 9670/9823 11 019 4.92 5.97 4.01
L Follicular9690,9691, 9695,9698 4 881 2.19 2.1 2.26
L BDGC9675, 9678, 9679, 9680, 9684 8 538 3.81 4.06 3.57
L Zona Marginal 9689, 9699, 9764 950 0.42 0.40 0.45
Enfermedades Immunoproliferativas
9671, 9760, 9761, 9762 1859 0.83 1.00 0.67
L de las c. del Manteau9673
1012 0.45 0.64 0.27
ICD-0-3 code N° de casos All Hombres Mujeres
L de celulas B maduras 42 855 19.14 21.30 17.07
Mieloma 9732 12 192 5.44 5.85 5.06
Leucemia de Plasmocytos 9733 92 0.04 0.04 0.05
Plasmacytoma 9731, 9734 1172 0.52 0.58 0.47
Burkitt L/L 9687, 9826 488 0.22 0.31 0.13
Leucemia B 9833, 9940 652 0.29 0.46 0.13
by age
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
1
2
3
4
5
6
7
8North
UK
East
Central
South
Year of diagnosis
Cru
de
Inci
den
ce R
ates
x 1
00,0
00
Leucemia linfoide Cronica
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0.45
0.5
0.55
0.6
0.65
0.7
0.75
0.8
0.85
0.9
0.95
Lymphoplasmacytic
Lymphoblastic Lymphoma
Burkitt
T lymphoma cutaneous
Other T cell lymphomas
Other specified NHL
Mantle cell/centrocytic
Marginal zone B cell/MALT mucose associated
Composite Hodgkin and NHL
Year of diagnosis
Evolución de la incidencia por tipo
Données RHEMCO 1980-2005
0
0,5
1
1,5
2
2,5
3
3,5
4
1980-1984 1985-1989 1990-1994 1995-1999 2000-2005
L. Lymphoplasmocytaires L. du ManteauL. Folliculaire L. B diffus à grandes cellulesL. de Burkitt L. de la zone marginaleLNH T
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
2
4
6
8
10
12
14
16
18
20
hodgkin disease
non hodgkin lymphoma
Year of diagnosis
Cru
de
In
cid
en
ce
Ra
tes
x 1
00
,00
0
Année
Ta
ux s
tan
da
rdis
és m
on
de
(é
ch
elle
lo
g)
1980 1985 1990 1995 2000 2005 2010
15
10
50
Homme
15
10
50
Année
Ta
ux s
tan
da
rdis
és m
on
de
(é
ch
elle
lo
g)
1980 1985 1990 1995 2000 2005 2010
15
10
50
Femme
15
10
50Incidence estimée Homme
Incidence estimée FemmeMortalité estiméeMortalité observée
Scenario= A ==> Figure 3: Tendance chronologique pour la France
Incidence and mortality estimates in France by NHL
1980-2005 : +2.7% /y in men +2.9%/y in women2000-2005 : -0.1%/y in men +0.4%/y in women
Level 1 Level 2 Level 3 level 4 t(8;21) inv(16) t(15;17) recurrent genetic abnormalities t(9;11) t(6;9) inv(3) or t(3;3) t(1;22) mutated NPM1 mutated CEBPA with multilineage disorders AML-MDS therapy related
Myeloid Haemopathies Acute Myeloid Leukaemia Minimaly differentiated without maturation with maturation myelomonocytic AML NOS monocytic/monoblastic erythoblastic megakaryoblastic basophilic A panmyelosis with myelofibrosis myeloid sarcoma M Prol with Down Syndrome Myeloid leuk Blastic plasmacytoid dendritic cell AL of ambiguous lineage CML Polycythemia vera Essential Thrombocytemia Chronic idiopathic myelofibrosis
Myeloproliferative neoplasms Systematic mastocytosis Mastocytosis Mast cell leukemia Mast cell sarcoma others Refractory Anaemia Refractory neutropenia Refractory thrombocytopenia RA with ring sideroblasts
Myelodysplastic syndromes RA with excess of blasts 5q- syndrome R Cytopenia with multilin. Dysplasia Others CMML Myelodysplastic/ JCMML Myeloproliferative neoplasms atypical CML others Prolif with PDGFRA, PDGFRB or FGFR1
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
1
2
3
4
5
6
Leucemia mieloide aguda North
UK
East
Central
South
Year of diagnosis
Cru
de
Inci
den
ce R
ates
x 1
00,0
00
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
0.5
1
1.5
2
2.5
3
Leucemia mieloide cronica North
UK
East
Central
South
Year of diagnosis
Cru
de
Inci
den
ce R
ates
x 1
00,0
00
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 20020
0.5
1
1.5
2
2.5
3
3.5
4
4.5
5
Leucemias
Leukemia NOS
Lymphatic Leukemia NOS
Acute Myeloid Leukemia
Chronic myeloprolipherative disorders
Mature B cell Leukemia
Mature cell T/NK cell Leukemia
Acute (precursor cell) Lymphatic Leukemia
Myelodisplastic syndrome
Cru
de
Inci
den
ce R
ates
x 1
00,0
00
Dr Milena SantDr Claudia AllemaniDr Carmen TereanuDr Franco Berrino
Istituto dei tumoriMilan, Italia
Dr Roberta De AngelisDr Roberto Capocaccia
Ariana Simonetti
Istituto Superiore di SanitàRoma, Italia
Dr Rafael Marcos GrageraGirona, Espana
Pr Marc MaynadiéDijon, Francia
Dr Otto VisserHolanda
Dr Jean Michel LutzNicer, Suissa
The HAEMACARE Group