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Coding Haematological Malignancies in Cancer Registries part II : Lymphoid malignancies Pr Marc Maynadié Registre des Hémopathies Malignes de Côte d'Or.

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1 Coding Haematological Malignancies in Cancer Registries part II : Lymphoid malignancies Pr Marc Maynadié Registre des Hémopathies Malignes de Côte d'Or Faculté de Médecine de Dijon

2 Definition Lymphoid malignancies = « Lymphoma » – proliferation developped from a lymphoid cell, – initiated in a lymphoid organ : Nodal extra nodal

3 Cellule souche totipotente Cellule souche lymphoïde Cellule souche myéloïde (CFU-GEMM) Phase centrale de différenciation indépendante pro B pré B B immature B mature naïve centroblastes centrocytes PlasmocytesLymphocyte B mémoire Activation Phase périphérique de différenciation Prothymocyte Thymocyte cortical CD3+ CD1+ Thymocyte cortical CD4+ CD8+ Thymocytes médullaires matures CD8+ LT CD8 "cytotoxique" LT CD4 "helper" LT mémoire CD4+ M O E L L E T H Y M U S M.O. Foll. I Sang Foll. II M.O. Org L. Sang Org L. II

4 Lymphoid territories

5 Lymph nodes

6 Lymph node: small ball-shaped organ of the immune system distributed widely throughout the body and linked by lymphatic vessels.

7 Zone Sombre Zone claire Manteau Zone marginale T B émergeant B recirculant B-blaste Cc Cb B mémoire Apoptose CDF Plasmocyte IgD IgM IgG/A/E CD23 CD39 CD10 CD38 CD77 Bcl2 Ki67 Bcl6 Mutations somatiques Commutation isotypique CD138

8 Precursor B Marrow Naive B cell B Marrow Follicle I LNH marginal zone (SLVL) Immunoblast Large B-cell NHL Paracortical area lympho- plasmocytic cell Lymphoplasmocytic NHL Medullar area Plasmocytes Marrow Blaste B Centroblast Centrocyte Marginal zone cell Memory cell Mantle cell Myeloma Follicular lymphomasBurkitt Diffuse Large B-cell NHL LAL LLC/SLL Mantle Dark areaClear zone CD19+ CD10+ Tdt+ cµ CD19+ CD23+ CD5+ sIgM-D CD38+ unmutated CD19+ CD5+ CD23- sIgM-D cµ CD23- CD77+ CD10+ CD10- CD77- CD23+ IgM IgM, G bcl2 CD39+ CD10- CD77- IgG, A CD20- CD138+ CD38+ Mutations sIgM-D CD19+ CD5+ CD23+ CD38- cIgM, CD20 Ag Mantle LNH Secondary follicle : germinal center

9 Very complex system: – Lineages: B, T, NK – Organs: primary and secondary lymphoid organs – Steps of differentiation Explain the numerous entities and changes in the classification All now grouped under the term « Lymhoma »

10 Epidemiology Incidence : – Very important increase since mid 1970s in NHL USA : +75% since 1975 Northern Europe : x2 France: x2,5 – In both sexes – Risk factors mainly unknown : virus, immunodeficiencies,… multifactorial process – Stabilisation since 2000

11 Panama 6,6 France : 13,3

12 Panama: 0,1 France: 2,43

13 Non Hodgkin Lymphoma Hodgkin Lymphoma Multiple Myeloma

14 Incidence and mortality estimates in France by NHL 1980-2005 : +2.7% /y in men +2.9%/y in women 2000-2005 : -0.1%/y in men+0.4%/y in women

15 Incidence and mortality estimates in France by Hodgkin L

16 Urbain – Rural (WPS Incidence) Données RHEMCO 1980-2005 UrbainRural p < 0,001 Men9,25,4 Women5,93,7

17 Incidence rate by entities RHEMCO 1980-2005 MWTotalSex ratio Diffuse large B-cell L2,651,812,021,46 Follicular L.1,771,861,810,95 Mantle cell L.0,340,130,232,62 Marginal Zone L.0,610,430,511,42 Burkitt L.0,360,140,252,57 Lymphoplasmocytoid L0,580,350,461,66 T NHL1,560,861,181,81

18 from Morton et al., Blood, 2007 Proportion of different entities

19 Evolution of incidence by entities Données RHEMCO 1980-2005

20 RHEMCO 1980-2005 Incidence according to age of NHL âge moyen : H : 61,9 ± 0,7 F : 66,1 ± 0,7 p < 0,001 âge moyen : H : 61,9 ± 0,7 F : 66,1 ± 0,7***

21 Incidence and mortality in France according to age by Hodgkin L

22 Epidemiology Relative Survival : Non Hodgkin Lymphoma Women Men

23 Epidemiology Relative Survival : Multiple Myeloma Women Men

24 Relative Survival evolution NHL in USA from Pulte et al., 2000

25 Principle of classification of Lymphoid proliferations B lineage – Issued from immature cells : Precursor Acute lymphoblastic leukemia and Lymphoblastic lymphoma – Issued from matured cells Non Hodgkin lymphoma Hodgkin lymphoma T/NK lineage – Issued from immature cells : Precursor Acute lymphoblastic leukemia and Lymphoblastic lymphoma – Issued from matured cells T Non Hodgkin lymphoma Natural Killer cells proliferations

26 Principle of classification of Lymphoid proliferations Histiocytic and dendritic cells Post-transplant proliferations

27 Coding practice Pathology/Cytology report mandatory – Morphology code : ICD-O-3 Clinical chart mandatory – Topography code – Stage – Treatment – Follow up

28 Precursor lymphoid neoplasms Cytology : blast cells Immunophenotype : immature lymphoid B or T cells Morphology : ICD-O-3 Topography : C42.1 = Bone marrow Stage : no ICD-O-4

29 Leucémie lymphoblastique B9836/3 Lymphome lymphoblastique B 9728/3 Leucémie aiguë lymphoblastique T9837/3 Lymphome lymphoblastique T9729/3 Leucémie aiguë lymphoblastique, NOS9835/5 Lymphome lymphoblastique, NOS9727/3 Regroupement confirmé Seuil des blastes utilisé antérieurement non conservé Precursor Lymphoid Neoplasm 9837/3 9836/3 9835/3

30 B immature cells CD19+ CD10+ CD20+ µ chain cyt Kappa or Lambda chain s T Immature cells CD3 cyt/s ; TCR ; CD99 CD1, CD4-, CD8- CD1-, CD4+ and CD8+ CD4 + or CD8+ Precursor lymphoid neoplasms

31 B lymphoblastic leukemia /lymphoma 9836/3 or 9728/3 – B Lymphoblastic leukemia/lymphoma, NOS9811/3 – B Lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2)9812/3 – B Lymphoblastic leukemia/lymphoma with t(v; 11q23) (MLL)9813/3 – B Lymphoblastic leukemia/lymphoma with t(12;21)(p13;q22)9814/3 – B Lymphoblastic leukemia/lymphoma with hyperdiploïdy9815/3 – B Lymphoblastic leukemia/lymphoma with hypodiploïdy9816/3 – B Lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32)9817/3 – B Lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3)9818/3 T Lymphoblastic leukemia/lymphoma 9837/3 or 9729/3 9837/3 Precursor Lymphoid Neoplasm

32 Mature Neoplasms Morphology : ICD-0-3 Topography : – Nodal lymphoma, code C77.-. – Many lymph nodes, code C77.8 – Nodal but unknown location, code C77.9 – Extra-nodal: coding the organ Stage : Ann Arbor, others ICD-O-4

33 Morphology coding rules Avoid « NOS » codes as far as possible 9590/3 Lymphoma, NOS 9591/3 Non Hodgkin Lymphoma, NOS 9650/3 Hodgkin lymphoma, NOS 9680/3 Large B-cell lymphoma, NOS 9690/3 Follicular lymphoma, NOS 9702/3 T-cell lymphoma, NOS 9727/3 Lymphoblastic lymphoma, NOS

34 Chronic lymphocytic leukemia/small lymphocytic lymphoma9823/3 B-cell prolymphocytic leukaemia9833/3 Splenic marginal zone lymphoma9689/3 Hairy cell leukaemia9940/3 Splenic B-cell lymphoma/leukaemia, unclassifiable9591/3 – Splenic diffuse red pulp small B-cell lymphoma9591/3 – Hairy cell leukaemia-variant9591/3 Lymphoplasmacytic lymphoma9671/3 – Waldenström macroglobulinemia9761/3 Heavy chain diseases9762/3 – Alpha heavy chain disease9762/3 – Gamma heavy chain disease9762/3 – Mu heavy chain disease9762/3 Plasma cell myeloma9732/3 Solitary plasmacytoma of bone9731/3 Extraosseous plasmocytoma9734/3 B cell Non Hodgkin Lymphoma

35 CLL/SLL Hyperlymphocytosis > 4500L/mm 3 Presence of B-cells with the phenotype : CD19+, CD5+, CD23+, Ig low intensity Bone marrow aspirate Bone marrow biopsy Karyotype : iso 17p : usefull for pronostic and treatment

36 B prolymphocytic leukemia Hyperlymphocytosis >>> 4500L/mm 3 Presence of B-cells with proeminent nucleoli Non specific phenotype : CD19+, CD5+/-, CD23-, Ig high intensity Bone marrow aspirate Bone marrow biopsy Karyotype

37 CLLProL LHCLHCLvSLVLMLFL CD 19 CD 10 CD 20 CD 22 CD 23 CD 24 CD 5 CD 25 FMC-7 Ig s HC-2 CD 43 + - + +/- + -/= - + low (K>L)(MD) - + - + -/+ + -/+ - + - +-++-+-++++--+-++-+-++++-- +-++----++++-++----+++ + - + - -/+ + - +/- + ++ - + -/+ ++ + - + - ++ high (L>K)(D) - + ++ + - + - + ++ h -

38 Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)9699/3 Nodal marginal zone lymphoma9699/3 – Paediatric nodal marginall zone lymphoma9699/3 Follicular lymphoma9690/3 – Paediatric follicular lymphoma9690/3 Primary cutaneous follicle centre lymphoma9597/3 Mantle cell lymphoma9673/3 Diffuse large B-cell lymphoma (DLBCL), NOS9680/3 – T-cell histiocyte riche large B-cell lymphoma9688/3 – Primary DLBCL of the CNS9680/3 – Primary cutaneous DLBCL, leg type9680/3 – EBV positive DLBCL of the elderly9680/3 DLBCL associated with chronic inflammation9680/3 Lymphomatoid granulomatosis9766/3 Primary mediastinal (thymic) large B-cell lymphoma9679/3 Intravascular large B-cell lymphoma9712/3 ALK positive large B-cell lymphoma9737/3 B cell Non Hodgkin Lymphoma

39 Plasmoblastic lymphoma9735/3 Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease9738/3 Primary effusion lymphoma9678/3 Burkitt lymphoma9678/3 B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma9680/3 B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma9596/3 B-cell Non Hodgkin Lymphoma

40 Follicular Lymphoma t(14;18)(q32;q21) t(2 ;18) (p11 ;q21) t(18 ;22) (q21 ;q11) 90 % BCL-2 Apoptose mutated Mantle cell lymphoma t(11;14)(q13;q32)70 % BCL-1/CCND1 Cycle cellulaire Unmutated and mutated Marginal zone Lymphoma Trisomies du 3, du 12, du 18 del7q 6-10 %?? Mutated and unmutated MALT lymphoma t(11;18)(q21;q21) t(1;14)(p22;q32) 50 % API2/MLT apoptose BCL-10 Mutated

41 Lympho plasmocytoid lymphoma t(9;14)(p13;q32)50 % PAX-5(transcription) Différentiation/Prolif ération Mutated CLL/SLL Trisomy 12, del7q?? unmutated and mutated Large B-cell NHL Abnormalities 3q2735-45 % BCL-6 (transcription) Différentiation B Mutated Burkitt L t(8;14)(q24;q32) t(2 ;8) ( p11 ; q24) t(8 ;22) (q24 ;q11) 80 % c-MYC prolifération Mutated Hodgkin ????Mutated Anaplastic Lymphoma t(2;5)(p23;q35) and variants 60 % NPM/ALK tyrosine kinase (signalisation) -

42 Lymphomes de Hodgkin 30 % des lymphomes Caractéristiques communes : o développement ganglionnaire, surtout région cervicale o la plupart surviennent chez des adultes jeunes o présence d'un petit nombre de cellules grandes mono ou multinuclées : cellules de Hodgkin ou de Reed Stenberg o Cellules tumorales souvent entourées de lymphocyte T en rosette

43 Deux types d'affections : Hodgkin nodulaire avec des lymphocytes prédominants Hodgkin classique : 4 sous-types : Sclérose nodulaire à cellularité mixte riche en lymphocytes déplété en lymphocytes Phénotype des cellules malignes identiques

44 LH nodulaire avec lymphocytes prédominants 5 % des Hodgkin Prolifération de cellules B monoclonales caractérisées par des grandes cellules appelées Popcorn ou LH cells. o Synonyme : Paragranulome de Poppema-Lennert o patients masculins o adénopathie périphérique localisée le plus souvent Documents diagnostiques : o compte rendu Anatomo-pathologique o stades de la classification Ann-Arbor Pronostic : bon avec 80 % de survie à 10 ans pour les stades I, II 9843/3

45 Lymphomes de Hodgkin classiques Proliférations monoclonales de cellules B composées en proportions variables de cellules mononuclées de Hodgkin, de cellules plurinucléées de Reed-Sternberg, au sein d'un infiltrat réactionnel fait de petits lymphocytes, d'éosinophiles, de neutrophiles, d'histocytes, de plasmocytes, de fibroblastes et de fibres de collagène. Epidémiologie : o 95 % des Lymphomes de Hodgkin o pics entre 15-35 ans et après 70 ans o rôle de l'EBV encore discuté 9650/3

46 4 types : o Sclérose nodulaire (70 %)9663/3 atteinte médiastinale dans 80 % o Cellularité mixte (20-25 %)9652/3 surtout ganglions périphériques et rates o Riche en lymphocytes (5 %)9651/3 surtout ganglions périphériques o Déplété en lymphocytes (< 1 %)9653/3 souvent associée à HIV – Ganglions rétropéritoneaux

47 Précurseur T MoelleThymus Cortex zone sous capsulaire Progéniteur T Prothymocyte Tdt CD3c CD1 Θ Tdt CD3s CD1a CD5 CD7 Thymocyte corticaux Thymocytes communs Thymocytes médullaires TCR CD4 +CD8+ CD4CD8 Médullaire LAL / Lymphome lymphoblastique NK CD16, CD56, CD57 CD2, CD7, +/- CD8 KiR L T CD3 TCR 5 CD4-, CD8- Peau Rate Muqueuse Sang Ag surtout nodaux chez ladulte Cortex Lymphomes T Périphériques Organes lymphoïdes secondaires Immunité innée Immunité acquise

48 Mature T-cell and NK-cell neoplasms T-cell prolymphocytic leukemia9834/3 T-cell large granular lymphocytic leukemia9831/3 Chronic lymphoproliferative disorder of NK-cells9831/3 Aggressive NK cell leukemia9948/3 Systemic EBV positive T-cell lymphoproliferative disease of childhood9724/3 Hydroa vacciniforme-like lymphoma9725/3 Adult T-cell leukemia/lymphoma9827/3 Extranodal NK/T cell lymphoma, nasal type9719/3

49 Hepatosplenic T-cell lymphoma9716/3 Subcutaneous panniculitis-like T-cell lymphoma9708/3 Mycosis fungoides9700/3 Sezary syndrome9701/3 Primary cutaneous CD30 positive T-cell lymphoproliferative disorders – Lymphomatoid papulosis9718/1 – Primary cutaneous gamma-delta T-cell lymphoma9718/3 Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma9709/3 Primary cutaneous CD4 positive small/medium T-cell lymphoma9709/3 Peripheral T-cell lymphoma, NOS9702/3 Angioimmunoblastic T-cell lymphoma9705/3 Anaplastic large cell lymphoma, ALK positive9714/3 Anaplastic large cell lymphoma, ALK negative9702/3 Nature T-cell and NK-cell neoplasms

50 Leucémie Prolymphocytaire T Synonyme : Leucémie Lymphoïde Chronique T Prolifération de lymphocytes T de phénotype mur envahissant le sang, la moelle, les ganglions, la rate, le foie et la peau.

51 Eléments diagnostiques Numération formule sanguine : hyperlymphocytose Immunophénotype : lymphocytes T :CD3, CD2, CD7 CD1Θ CD4, 60 % CD4, CD8, 25 % CD4Θ, CD8, 15 % Biol. Mol. : TCR réarrangé Caryotype : anomalie en 14q11 et q32 Pronostic : mauvais – médiane de survie < 1 anS

52 Leucémie/lymphome T de l'Adulte (ATLL) 9827/3 Prolifération causée par le virus HTLV-1 Endémie : Sud Ouest Japon, Afrique Centrale, Caraïbes Contact avec virus très jeune longue latence Pléomorphisme cellulaire (Flower cells) 3 formes cliniques : smouldering, chronique, aiguë Phénotype : CD3, CD2, CD5 mais CD7 Θ, CD4 Pronostic : mauvais

53 Mycosis fongoïdes9700/3 Lymphome T cutané le plus fréquent (env. 50 % LNH cutanés) Infiltration cutanée progressive (tache, plaque, tumeur) Cellules CD4 Diagnostic : difficile surtout au début Pronostic : fonction de l'extension Diagnostic sur le CR anatomo-clinique +++

54 Syndrome de Sézary9701/3 LNH cutané T rare (< 5 %) Associe : o adénopathies o érythrodermie o cellules malignes cérébriformes monoclonales T dans le sang o > 1000 cellules de Sézary/mm3 dans le sang Phénotype : CD3, CD4, CD7Θ Pronostic : mauvais

55 Lymphomes T périphériques, NOS 9702/3 Ensemble hétérogène de lymphomes T matures, nodaux ou extranodaux qui ne correspondent à aucune entité décrite... Diagnostic par défaut Inclus : LNH anaplasique à grandes cellules, AlkΘ

56 LNH anaplasiques à grandes cellules Alk 9714/3 LNH T avec implication du gène Alk (chrom. 2) et expression de CD30 Diagnostics différentiels nombreux : o ALCL AlkΘ o ALCL cutanés primitifs o autres LNH B ou T avec traits anaplasiques ou exprimant CD30 Difficile - S'en tenir au CR anatomoclinique

57 Syndrome myéloprolifératif T cutanés primitifs CD30 9718/3 2 ème groupe de LNH cutanés T (30 %) Inclus : o LNH anaplasique à grandes cellules cutanés primitif o Papulose lymphomatoïde (non maligne) o Borderline

58 T CLLLPL TLG-L TLGL NKMF CD1a CD 2 CD 3 CD 5 CD 7 CD 4 CD 8 CD 25 CD 16 CD 56 CD 57 TCR + + (s) + + (65 %) + (21 %) 4-/8+ - + - + + (cyt) + - +/- - + - + - + - +/- + - -/+ + +/- - + +/- - < + < < (33 %) + - +

59 Histiocytic and dendritic cell neoplasms Histiocytic sarcoma9755/3 Langerhans cell histiocytosis9751/3 Langerhans cell sarcoma9756/3 Interdigitating dendritic cell sarcoma9757/3 Follicular dendritic cell sarcoma9758/3 Fibroblastic reticular cell tumour9759/3 Indeterminate dendritic cell tumour9757/3 Disseminated juvenile xanthogranuloma

60 Post-transplant lymphoproliferative disorders (PTLD) Early lesions – Plasmocytic hyperplasia9971/3 – Infectious mononucleosis-like PTLD9971/1 Polymorphic PTLD9971/3 Monomorphic PTLD (B- and T/NK-cell types)* Classical Hodgkin lymphoma type PTLD* *These lesions are classified according to the leukemia or lymphoma to which they correspond, and are assigned the respective ICD-0 code.

61 Mature Neoplasms Morphology : ICD-0-3 Topography : – Nodal lymphoma, code C77.-. – Many lymph nodes, code C77.8 – Nodal but unknown location, code C77.9 – Bone MarrowcodeC42.1 – Extra-nodal: coding the organ Stage : Ann Arbor, others ICD-O-4

62 Nodal areas Nodal, NOSC77.9 Multiple territories ( 2) C77.8 Pelvis C77.5 Inguinal and leg C77.4 Axillary and ArmC77.3 Intra-abdominal C77.2 Intra-thoracic C77.1 Head, neck and faceC77.0

63 Nodal Lymphoid areas Lingual tonsilsC02.4 Palatal tonsilsC09.9 Pharynx tonsilsC11.1 Waldeyers ringC14.2 IleumC17.2 AppendixC18.1 Thymus glandC37.9 SpleenC42.2

64 Extra nodal coding rules +/- 30% of NHL are extra nodal Stomach > small Intestine > Skin > Bone > CNS > Colon > Breast, … Unique involved organ : coding the specific topography : i.e. Stomach = C16.x more than one organ is involved :C77.8

65 Bone MarrowC42.1 Multiple lymph nodesC77.8 Unique (extra-lymphatic) organ : organ code Multiple organsC77.8 Lymph node(s) + Bone Marrow C42.1 Lymph node (s) + unique organ : organ code Lymph node(s) + multiples organs C77.8 Bone Marrow + organ(s) +/- lymph node C42.1 Topography coding rules Bone Marrow > multiple LN or organ > organ > lymph node

66 Mature Neoplasms Morphology : ICD-0-3 Topography : – Nodal lymphoma, code C77.-. – Many lymph nodes, code C77.8 – Nodal but unknown location, code C77.9 – Extra-nodal: coding the organ Stage : Ann Arbor, others ICD-O-4

67 Ann Arbor staging Usefull in NHL and in Hodgkin lymphoma

68 Ann Arbor staging Stage I - disease in single lymph node or lymph node region. Stage II - disease in two or more lymph node regions on same side of diaphragm. Stage III - disease in lymph node regions on both sides of the diaphragm are affected. Stage IV - disease is wide spread, including multiple involvement at one or more extranodal (beyond the lymph node) sites, such as the bone marrow. A: absence of constitutional symptoms; a: of biological symptoms B: presence of constitutinal symptoms; b: of biological symptoms E: the disease is "extranodal" (not in the lymph nodes) or has spread from lymph nodes to adjacent tissue. X: the largest deposit is >10 cm large ("bulky disease"), or whether the mediastinum is wider than 1/3 of the chest on a chest X-ray. S: the disease has spread to the spleen.

69 Multiple Myeloma Stage I : – Low monoclonal protein levels: IgG<50g/L ; IgA<30g/L; Urine BJ<4g/24h – No or solitary bone lesion – Normal Hb, serum calcium, Ig levels Stage II: overall between I and III Stage III: any of one or more of the following – High M-protein: IgG>70g/L; IgA>50g/L; Urine light chain >12g/L – Advanced, multiple lytic bone lesions – Hb 12mg/dL A: serum creatinine 2mg/dL

70 Chronic Lymphocytic Leukemia Binets stage Stage A : 2 or less lymph nodes involved Stage B: 3 or more lymph nodes involved Stage C: Hb<10g/dL or platelets<100,000/mm 3

71 Preguntas? Exercicios

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