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