内科学淋巴瘤邹善华.ppt

Definition：A group of neoplastic diseases of lymphoid tissues Classification： Hodgkins Disease, HD (Hodgkins Lymphoma) Non Hodgkin s Lymphoma,NHL),General Considerations,Clinical Features： Painless, progressive lymphadenectasis Fever, weight loss, night sweat；cachexia Hepatosplenomegaly Epidemiology： Incidence： in cities / in the country; male/female; age HD 811；NHL 90%,General Considerations,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,Immune States of Host： Immunodeficiency/immunosuppression Mechanism：Antigenic stimulation lymphadenosis ( modulation) infinite proliferation lymphoma,Etiology and Pathogenesis,Classification of Lymphoma,HD：Reed-Sternberg(R-S) Cell,Classification of Lymphoma,Rye Classification ，1966 Lymphocyte predominance Nodular sclerosis Mixed cellularity Lymphocyte depletion,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,Classification of Lymphoma,NHL：,Classification of Lymphoma,NHL： IWF，1982 REAL，1994 WHO，2001,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,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,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,Working Formulation,Miscellaneous composite mycosis fungoides histiocytic extramedullary plasmacytoma unclassifiable other,REAL Classification,B-cell neoplasms I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphoma II. Peripheral B-cell neoplasms 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,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,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,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,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,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,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,WHO Classification,2001,T-Cell and Natural Killer Cell Neoplasms I. Precursor T cell neoplasm: a.Precursor T-lymphoblastic lymphoma/leukemia b. Blastic NK lymphoma,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,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,Clinical Features,Lab and Other Findings,Peripheral Blood and Bone Marrow HD： Eosinophilia； RS 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,Diagnosis Painless,progressive lymphadenectasis Biopsy Skin Lesion Biopsy Bone Marrow Aspiration and Biopsy Biopsy of Other Sites,Diagnosis and Differential Diagnosis,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,Staging of lymphoma,Differential Diagnosis Lymphadenectasis： Chroniclymphadenitis Tuberculous lymphadenitis Tumour node metastases Fever：Septicaemia, Tuberculosis, Rheumatic disease Extranodal Lymphoma:Other malignant tumer,Diagnosis and Differential Diagnosis,Radiation Therapy 60Co,accelerator linear Dosage:3040GY，34W Methods： L