内科学 淋巴瘤-邹善华

1、Definition：A group of neoplastic diseases of lymphoid tissues Classification： Hodgkins Disease, HD (Hodgkins Lymphoma) Non Hodgkin s Lymphoma,NHL) Clinical Features： Painless, progressive lymphadenectasis Fever, weight loss, night sweat；cachexia Hepatosplenomegaly Epidemiology： Incidence： in cities

2、/ in the country; male/female; age HD 811；NHL 90%Virulogy: EB virusBurkitt lymphoma, HD Human T-cell Lymphotropic Virus type 1, HTLV-1 Adult T-cell lymphoma/leukemia , , ATL HTLV-2Mycosis Fungoides Helicobacter PyloriMALT lymphoma Kaposi sarcoma-associated herpesvirus primary body cavity lymphoma Im

3、mune States of Host： Immunodeficiency/immunosuppressionMechanism：Antigenic stimulation lymphadenosis ( modulation) infinite proliferation lymphomaHD：Reed-Sternberg(R-S) Cell Rye Classification ，1966 Lymphocyte predominance Nodular sclerosis Mixed cellularity Lymphocyte depletion WHO Classification,

4、2001 Nodular lymphocyte predominance Hodgkins lymphoma Classical Hodgkins lymphoma Lymphocyte-rich classical Hodgkins lymphomaNodular sclerosis Hodgkins lymphoma Mixed cellularity Hodgkins lymphoma Lymphocyte depletion Hodgkins lymphomaNHL：NHL： IWF，1982 REAL，1994 WHO，2001Low-grade lymphomaLow-grade

5、lymphomaA. small lymphocytic, consistent with A. small lymphocytic, consistent with chronic lymphocytic leukemia chronic lymphocytic leukemia plasmacytoidplasmacytoid B. follicular, predominantly small cleaved cell B. follicular, predominantly small cleaved cell diffuse areas diffuse areas sclerosis

6、 sclerosis C.C. follicular, mixed small cleaved and large cell follicular, mixed small cleaved and large cell diffuse areas diffuse areas sclerosis Intermediate-grad lymphomaIntermediate-grad lymphomaD.D. follicular, predominantly large cell follicular, predominantly large cell diffuse areas diffuse

7、 areas sclerosis sclerosis E. diffuse, small cleaved cell E. diffuse, small cleaved cell sclerosis sclerosis F.F. diffuse, mixed small and large cell diffuse, mixed small and large cell sclerosis sclerosis epithelioidepithelioid cell component cell component G.G. diffuse, large cell diffuse, large c

8、ell cleaved cell cleaved cell noncleavednoncleaved cell cell sclerosis sclerosis High-grade lymphomaHigh-grade lymphomaH. H. mmunoblasticmmunoblastic, large cell, large cell plasmacytoidplasmacytoid clear cell clear cell polymorphous polymorphous epithelioidepithelioid cell component cell component

9、I. lymphoblastic, convoluted or I. lymphoblastic, convoluted or nonconvolutednonconvoluted cell cell J. small noncleaved cell, Burkitts or non-Burkitts Miscellaneous composite mycosis fungoides histiocytic extramedullary plasmacytoma unclassifiable otherlB-cell neoplasmsI. Precursor B-cell neoplasm:

10、 precursor B-lymphoblastic I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphomaleukemia/lymphomaII. Peripheral B-cellII. Peripheral B-cell n neoplasms A. B-cell chronic lymphocytic leukemia/A. B-cell chronic lymphocytic leukemia/prolymphocyticprolymphocytic leukemia/small leukem

11、ia/small lymphocyticlymphomalymphocyticlymphoma B. B. LymphoplasmacytoidLymphoplasmacytoid lymphoma/ lymphoma/immunocytomaimmunocytoma C. Mantle cell lymphomaC. Mantle cell lymphoma D. Follicle center cell D. Follicle center cell lymphoma,follicularlymphoma,follicular 1. Provisional 1. Provisional c

12、ytologiccytologic grades: grades: I small cell, I small cell, II mixed small and large cell, II mixed small and large cell, III large cell III large cell 2. Provisional subtype: diffuse, predominantly 2. Provisional subtype: diffuse, predominantly small cell type small cell type E. Marginal zone B-c

13、ell lymphomaE. Marginal zone B-cell lymphoma 1. 1. ExtranodalExtranodal (MALT-type (MALT-type +/- +/- monocytoidmonocytoid B cells) B cells) 2. Provisional subtype: nodal (+/- 2. Provisional subtype: nodal (+/- monocytoidmonocytoid B cells) B cells) F. Provisional entity: F. Provisional entity: sple

14、nicsplenic marginal zone lymphomamarginal zone lymphoma (+/- villous lymphocytes) (+/- villous lymphocytes) G. Hairy cell leukemia G. Hairy cell leukemia H. H. PlasmacytomaPlasmacytoma/plasma cell myeloma /plasma cell myeloma I. Diffuse large B-cell lymphoma I. Diffuse large B-cell lymphoma 1. Subty

15、pe: primary 1. Subtype: primary mediastinalmediastinal ( (thymicthymic) B-cell ) B-cell lymphoma lymphoma J. J. BurkittsBurkitts lymphoma lymphoma K. Provisional entity: high-grade B-cell lymphoma, K. Provisional entity: high-grade B-cell lymphoma, BurkittBurkitt-like-like T-cell and putative NK-cel

16、l T-cell and putative NK-cell neoplasmsneoplasms I. Precursor T-cell neoplasm: precursor T- I. Precursor T-cell neoplasm: precursor T- lymphoblastic lymphoma/leukemia lymphoblastic lymphoma/leukemia II. Peripheral T-cell and NK-cell II. Peripheral T-cell and NK-cell neoplasmsneoplasms A. T-cell chro

17、nic lymphocytic leukemia / A. T-cell chronic lymphocytic leukemia / prolymphocyticprolymphocytic leukemia leukemia B. Large granular lymphocyte leukemia B. Large granular lymphocyte leukemia 1. T-cell type 2. NK-cell type 1. T-cell type 2. NK-cell type C. Mycosis C. Mycosis fungoides/Sezarysyndromef

18、ungoides/Sezarysyndrome D. Peripheral T-cell D. Peripheral T-cell lymphomas,unspecifiedlymphomas,unspecified 1. Provisional 1. Provisional cytologiccytologic categories: categories: medium-sized cell, mixed medium and medium-sized cell, mixed medium and large cell, large cell, large cell, large cell

19、, lymphoepithelioidlymphoepithelioid cell cell 2. Provisional subtype: 2. Provisional subtype: hepatosplenichepatosplenic gamma/delta T-cell lymphoma gamma/delta T-cell lymphoma 3. Provisional subtype: 3. Provisional subtype: subcutaneous subcutaneous panniculiticpanniculitic T-cell T-cell lymphoma

20、lymphoma E. E. AngioimmunoblasticAngioimmunoblastic T cell lymphoma T cell lymphoma F. F. AngiocentricAngiocentric lymphoma lymphoma G. Intestinal T-cell lymphoma G. Intestinal T-cell lymphoma (+/- (+/- enteropathyenteropathy associated) associated) H. Adult T-cell lymphoma/leukemia H. Adult T-cell

21、lymphoma/leukemia I. I. AnaplasticAnaplastic large cell lymphoma large cell lymphoma 1. CD30+ -cell type 1. CD30+ -cell type 2. T-cell type 2. T-cell type 3. Null-cell types 3. Null-cell types J. Provisional entity: J. Provisional entity: anaplasticanaplastic large cell large cell lymphoma, Hodgkins

22、-likelymphoma, Hodgkins-like B-cell neoplasms lI. Precursor B-cell neoplasm: a. Precursor B-lymphoblastic leukemia/lymphomalymphoblastic leukemia/lymphomalII. Mature (peripheral) B-cell neoplasms l a B-cell chronic lymphocytic leukemia/small lymphocytic chronic lymphocytic leukemia/small lymphocytic

23、 lymphoma lymphoma l b B-cell prolymphocytic leukemia l c c LymphoplasmacyticLymphoplasmacytic lymphoma lymphoma l d Spenic marginal zone B-cell lymphoma (+/- villous lymphocytes) l e Nodal marginal zone lymphoma(+/- monocytoid B- cells) l f Extranodal marginal zone B-cell lymphoma of mucosa-associa

24、ted lymphoid tissue (MALT) type l g Hairy cell leuekmia l h Plasma cell myeloma/plasmacytoma l i Follicular lymphoma, follicle center l j Mantle cell lymphoma l k Diffuse large cell B-cell lymphomaMediastinal large B-cell lymphoma Intravascular large B-cell lymphoma Primary effusion lymphoma l l Bur

25、kitts lymphoma/Burkitts cell leukemia lB-cell proliferations of uncertain malignant potential l Lymphomatoid granulomatosis l Post-transplant lymphoproliferative disorder T-Cell and Natural Killer Cell Neoplasms lI. Precursor T cell neoplasm: a.Precursor T-lymphoblastic lymphoma/leukemia b. Blastic

26、NK lymphoma II. Mature (peripheral) T cell and NK-cell neoplasms l a T cell prolymphocytic leukemia l b T-cell granular lymphocytic leukemia l c Aggressive NK Cell leukemia l d Adult T cell lymphoma/leukemia (HTLV1+) l e Extranodal NK/T-cell lymphoma, nasal type l f Enteropathy-type T-cell lymphoma

27、l g Hepatosplenic gamma-delta T-cell lymphoma l h Subcutaneous panniculitis-like T-cell lymphoma l i Mycosis fungoides/Sezarys syndrome l j Primary Cutaneous Anaplastic large cell lymphoma T/null cell l k Peripheral T cell lymphoma, unspecified l l Angioimmunoblastic T cell lymphomal m Primary Syste

28、mic Anaplastic large cell lymphoma, T/null cell T-cell proliferation of uncertain malignant potential l Lymphomatoid papulosis HDNHL Age Young people More often in middle-aged people Superficial lymph nodes 6080 50% (first observed as tumefaction of cervical or supraclavicular lymph nodes) Mediastin

29、al lymph nodes 50% 20% Retroperitoneal lymph nodes Seldom invaded Often invaded Fever Often occurred Seldom in early period Itch of skin Often occurred in some young female patients Seldom occurred Specific skin lesion Seldom occurred Often occurredExtranodal sites Seldom occurred Often occurred (Wa

30、ldeyers ring, gastrointestinal tract, bone marrow, CNS, liver, lung, pleura,bone, skin, kidney) Way of dissemination Metastasis to nearby lymph nodes In a jumping way Bone marrow involvement Seldom occurred Often occurred Progress Slower Faster Prognosis Better Worse Peripheral Blood and Bone Marrow

31、 HD： Eosinophilia； RS cell NHL：Lymphocytosis; Complicated leukemiaLDH，2 -MG , Coombs test，globulinRadiologic studies Chest X-ray CT, MRIRadioactive nuclidePET-CTB-ultrasoundBiopsyImmunophenotype Cytogenetic and Molecular Studies Diagnosis Painless,progressive lymphadenectasis Biopsy Skin Lesion Biop

32、sy Bone Marrow Aspiration and Biopsy Biopsy of Other Sites Stage Definition Involvement of a single lymph node region or of a single Involvement of a single lymph node region or of a single extranodalextranodal organ or organ or site(Esite(E) ) Involvement of two or more node regions on the same sid

33、e Involvement of two or more node regions on the same side of the of the diaphragm,ordiaphragm,or localized involvement of an localized involvement of an extranodalextranodal site or site or organ(Eorgan(E ) and one or more lymph ) and one or more lymph node regions on the same side of the diaphragm

34、 node regions on the same side of the diaphragm Involvement of lymph node regions on both sides of the Involvement of lymph node regions on both sides of the diaphragm which may also be accompanied by localized diaphragm which may also be accompanied by localized involvement of an involvement of an

35、extranodalextranodal organs or organs or site(Esite(E ) or ) or spleen (s ) or both ( spleen (s ) or both (sEsE ) ) Diffuse or disseminated involvement of one or more distant extranodal organs with or without associated lymph node involvementB symptoms Fever 38 ,night sweats,and/or weight loss 10% of body in the 6 months Staging of lymphomaStaging of lymphomaDifferential DiagnosisDifferential Diagnosis Lymphadenectasis： Chroniclymphadenitis Tuberculous lym