内科学 淋巴瘤-邹善华

单击此处编辑母版标题样式,*,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,Lymphoma,Zhongshan Hospital ,Fudan UniversityZou Shanhua,1,D,efinition,：,A group of neoplastic diseases of lymphoid tissues,Classification,：,Hodgkin,s Disease, HD,(Hodgkin,s Lymphoma),Non Hodgkin,s Lymphoma,NHL),General Considerations,2,Clinical Features,：,Painless, progressive lymphadenectasis,Fever, weight loss, night sweat,；,cachexia,Hepatosplenomegaly,Epidemiology,：,Incidence,：,in cities / in the country;,male/female; age,HD 8,11,；,NHL 90%,General Considerations,3,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,Etiology and Pathogenesis,4,Immune States of Host,：,Immunodeficiency/immunosuppression,Mechanism,：,Antigenic stimulation ,lymphadenosis ( modulation,), infinite proliferation,lymphoma,Etiology and Pathogenesis,5,Classification of,Lymphoma,HD,：,Reed-Sternberg(R-S) Cell,6,Classification of,Lymphoma,Rye Classification,，,1966,Lymphocyte predominance,Nodular sclerosis,Mixed cellularity,Lymphocyte depletion,7,Classification of,Lymphoma,WHO Classification, 2001,Nodular lymphocyte predominance Hodgkins lymphoma,Classical Hodgkins lymphoma,Lymphocyte-rich classical Hodgkins lymphoma,Nodular sclerosis Hodgkins lymphoma,Mixed cellularity Hodgkins lymphoma,Lymphocyte depletion Hodgkins lymphoma,8,Classification of,Lymphoma,NHL,：,9,Classification of,Lymphoma,NHL,：,IWF,，,1982,REAL,，,1994,WHO,，,2001,10,Working Formulation,Low-grade lymphoma,A. small lymphocytic, consistent with,chronic lymphocytic leukemia,plasmacytoid,B. follicular, predominantly small cleaved cell,diffuse areas,sclerosis,C.,follicular, mixed small cleaved and large cell,diffuse areas,sclerosis,11,Working Formulation,Intermediate-grad lymphoma,D.,follicular, predominantly large cell,diffuse areas,sclerosis,E. diffuse, small cleaved cell,sclerosis,F.,diffuse, mixed small and large cell,sclerosis,epithelioid cell component,G.,diffuse, large cell,cleaved cell,noncleaved cell,sclerosis,12,Working Formulation,High-grade lymphoma,H. mmunoblastic, large cell,plasmacytoid,clear cell,polymorphous,epithelioid cell component,I. lymphoblastic, convoluted or nonconvoluted cell,J. small noncleaved cell, Burkitts or non-Burkitts,13,Working Formulation,Miscellaneous,composite,mycosis fungoides,histiocytic,extramedullary plasmacytoma,unclassifiable,other,14,REAL Classification,B-cell neoplasms,I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphoma,II. Peripheral B-cell,n,eoplasms,A. B-cell chronic lymphocytic leukemia/prolymphocytic leukemia/small lymphocyticlymphoma,B. Lymphoplasmacytoid lymphoma/immunocytoma,C. Mantle cell lymphoma,D. Follicle center cell lymphoma,follicular,1. Provisional cytologic grades:,I small cell,II mixed small and large cell,III large cell,2. Provisional subtype: diffuse, predominantly,small cell type,15,REAL Classification,E. Marginal zone B-cell lymphoma,1. Extranodal (MALT-type +/- monocytoid B cells),2. Provisional subtype: nodal (+/- monocytoid B cells),F. Provisional entity: splenic,marginal zone lymphoma,(+/- villous lymphocytes),G. Hairy cell leukemia,H. Plasmacytoma/plasma cell myeloma,I. Diffuse large B-cell lymphoma,1. Subtype: primary mediastinal (thymic) B-cell lymphoma,J. Burkitts lymphoma,K. Provisional entity: high-grade B-cell lymphoma, Burkitt-like,16,REAL Classification,T-cell and putative NK-cell neoplasms,I. Precursor T-cell neoplasm: precursor T-,lymphoblastic lymphoma/leukemia,II. Peripheral T-cell and NK-cell neoplasms,A. T-cell chronic lymphocytic leukemia /,prolymphocytic leukemia,B. Large granular lymphocyte leukemia,1. T-cell type 2. NK-cell type,C. Mycosis fungoides/Sezarysyndrome,17,REAL Classification,D. Peripheral T-cell lymphomas,unspecified 1. Provisional cytologic categories:,medium-sized cell, mixed medium and,large cell, large cell,lymphoepithelioid cell,2. Provisional subtype: hepatosplenic,gamma/delta T-cell lymphoma,3. Provisional subtype:,subcutaneous panniculitic T-cell,lymphoma,E. Angioimmunoblastic T cell lymphoma,F. Angiocentric lymphoma,18,REAL Classification,G. Intestinal T-cell lymphoma,(+/- enteropathy associated),H. Adult T-cell lymphoma/leukemia,I. Anaplastic large cell lymphoma,1. CD30+ -cell type,2. T-cell type,3. Null-cell types,J. Provisional entity: anaplastic large cell lymphoma, Hodgkins-like,19,WHO Classification,2001,B-cell neoplasms,I. Precursor B-cell neoplasm:,a. Precursor B-,lymphoblastic leukemia/lymphoma,II. Mature (peripheral) B-cell neoplasms,a B-cell,chronic lymphocytic leukemia/small lymphocytic,lymphoma,b B-cell prolymphocytic leukemia,c Lymphoplasmacytic lymphoma,d Spenic marginal zone B-cell lymphoma (+/- villous,lymphocytes),e Nodal marginal zone lymphoma(+/- monocytoid B-,cells),f Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type,20,WHO Classification,2001,g Hairy cell leuekmia,h Plasma cell myeloma/plasmacytoma,i Follicular lymphoma, follicle center,j Mantle cell lymphoma,k Diffuse large cell B-cell lymphoma,Mediastinal large B-cell lymphoma,Intravascular large B-cell lymphoma,Primary effusion lymphoma,l Burkitts lymphoma/Burkitts cell leukemia,B-cell proliferations of uncertain malignant potential,Lymphomatoid granulomatosis,Post-transplant lymphoproliferative disorder,21,WHO Classification,2001,T-Cell and Natural Killer Cell Neoplasms,I. Precursor T cell neoplasm:a.Precursor T-lymphoblastic,lymphoma/leukemiab. Blastic NK lymphoma,22,WHO Classification,2001,II. Mature (peripheral) T cell and NK-cell neoplasms,a T cell prolymphocytic leukemia,b T-cell granular lymphocytic leukemia,c Aggressive NK Cell leukemia,d Adult T cell lymphoma/leukemia (HTLV1+),e Extranodal NK/T-cell lymphoma, nasal type,f Enteropathy-type T-cell lymphoma,g Hepatosplenic gamma-delta T-cell lymphoma,h Subcutaneous panniculitis-like T-cell lymphoma,i Mycosis fungoides/Sezarys syndrome,23,WHO Classification,2001,j Primary Cutaneous Anaplastic large cell,lymphoma T/null cell,k Peripheral T cell lymphoma, unspecified,l Angioimmunoblastic T cell lymphoma,m Primary Systemic Anaplastic large cell,lymphoma, T/null cell,T-cell proliferation of uncertain malignant potential,Lymphomatoid papulosis,24,Clinical Features,25,HD,NHL,Age,Young people,More often in middle-aged people,Superficial lymph nodes,60,80,50%,(first observed as tumefaction of cervical or supraclavicular lymph nodes),Mediastinal 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 occurred,Extranodal sites,Seldom occurred,Often occurred,(Waldeyers 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,26,Lab and Other Findings,Peripheral Blood and Bone Marrow,HD,：,Eosinophilia,；,R,S cell,NHL,：,Lymphocytosis; Complicated leukemia,LDH,，,2 -MG , Coombs test,，,globulin,Radiologic studies,Chest X-ray,CT, MRI,Radioactive nuclide,PET-CT,B-ultrasound,Biopsy,Immunophenotype,Cytogenetic and Molecular Studies,27,Diagnosis,Painless,progressive lymphadenectasis Biopsy,Skin Lesion Biopsy,Bone Marrow Aspiration and Biopsy,Biopsy of Other Sites,Diagnosis and Differential Diagnosis,28,Staging of lymphoma,Stage Definition, Involvement of a single lymph node region or of a single extranodal organ or site(E), Involvement of two or more node regions on the same side,of the diaphragm,or localized involvement of an,extranodal site or organ(E ) and one or more lymph,node regions on the same side of the diaphragm, Involvement of lymph node regions on both sides of the,diaphragm which may also be accompanied by localized,involvement of an extranodal organs or site(E ) or,spleen (s ) or both (sE ),Diffuse or disseminated involvement of one or more distant,extranodal organs with or without associated lymph node involvement,B symptoms Fever,38,night sweats,and/or weight loss,10% of body in,the 6 months,29,Staging of lymphoma,30,Differential Diagnosis,Lymphadenectasis,：,Chroniclymphadenitis,Tuberculous lymphadenitis,Tumour node metastases,Fever,：,Septicaemia, Tuberculosis,Rheumatic disease,Extranodal Lymphoma:Other malignant tumer,Diagnosis and Differential Diagnosis,31,Radiation Therapy,60,Co,accelerator linear,Dosage:30,40GY,，,3,4W,Methods,：,Local field irradiation(LFI),Mantle or inverted Y irradiation,Total nodal irradiation(TNI),Treatment,32,Treatment Fields,33,Indication,：,HD A,A,：,Extended field,irradiation(EFI),B,B ,A,：,TNI and/or combination,chemotherapy,B ,：,Combination chemotherapy mainly,( or add local irradiation),NHL ,(,Indolent), (,Aggressive),：,EFI or local,irradiation,、,：,Combination chemotherapy mainly,( or add local irradiation),Treatment,34,Chemotherapy,HD,：,MOPP,ABVD,NHL,：,Indolent: chlorambucil, CTX; COP,Aggressive: CHOP, m-BACOD, MACOP-B,Relapsed: ESHAP,Treatment,35,Interferon,Anti-CD20 Monoclonal Antibody,Transplantation 0r Haemopoietic Stem Cell,Operation,Treatment,36,1,、,HD,：,Histology,clinical stage,2,、,NHL,：,Age,*,Stage,*,B symptoms,Performance status,*,Mass size,Number of extranodal sites,*,Bone marrow involvement,LDH,*,2 -MG,Histology,International Prognostic IndexIPI,Prognosis,37,