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1、The Diseases of Hematopoietic and lymphoid systems,1,PPT学习交流,Structure and function,Hematopoietic and lymphoid system is composed of myeloid tissue(bone marrow) and lymphoid tissues(thymus,spleen,lymph nodes and extranode lymphoid tissues). The thymus and bone marrow are often termed central lymphoi

2、d tissues The remaining organs are actively involved in the immune response,and constitute the peripheral lymphoid tissue.,2,PPT学习交流,3,PPT学习交流,4,PPT学习交流,5,PPT学习交流,6,PPT学习交流,Diseases,Infection and reactive proliferations The lymphoid neoplasmas Myeloid neoplasms Histocytic neoplasms,7,PPT学习交流,Lymphoi

3、d neoplasms,All lymphoid neoplasms are derived from a single transformed cell and are therefore monoclonal. Lymphoid neoplasms are a group of tumors that their clinical manifestations and behavior vary widely. They are classified as non-Hodgkins lymphoma(NHLs)and Hodgkinss lymphoma based on the feat

4、ures of tumor cells. NHLs account for about 70% to 8014) myc-Ig follicular lymphoma: t(14;18) bcl-2-Ig CLL:Philadelphia chromosome t(9,22) c-abl,bcr,16,PPT学习交流,Etiology(3),3.Autoimmune& immunodeficieny Disease eg. Receptor of transplanted organs. AIDS patients,17,PPT学习交流,lymphoma,18,PPT学习交流,Leukemia

5、 / Lymphoma,These are high-grade NHLs composed of diffuse sheets of medium-size lymphoid cells.They may be B- or T-cell lineage. These aggressive tumors affect predominantly in children,accounting for 80% of childhood leukemia. The pre-B-cell lymphoma mainly affects children,but the Pre-T-cell tumor

6、s mainly affect adolescent males.,19,PPT学习交流,Morphology,Mircoscopically,the lymph nodes affected by neoplastic cells are replaced by small to medium-sized blastcells with scant cytoplasm and inconspicuous nucleoli. In blood smear slide,the nuclei of lymphoblasts with Wright-Giemsa staining show some

7、what coarse and clumped chromatin and one or two nucleoli.,20,PPT学习交流,Blood and bone marrow changes,In peripheral blood,the white cell count is usually increased.sometimes more than 100,000/ul. Anemia is almost present. The platelet count is ususlly depressed to less than 100,000/ul. Bone pain and t

8、enderness result from marrow expansion with infiltration of the subperiosteum. Splenomegaly.,21,PPT学习交流,Immunophenotype and karyotype,TdT present in more than 95% of cases CD19(B cell marker) CD2(T cell marker),22,PPT学习交流,Clinical features,The manifestations in these diseases are similar to that of

9、AML. Anemia,hemorrhage and infection as well as related symptoms,23,PPT学习交流,Chronic lymphocytic leukemia(CLL)/small lymphocytic lymphoma(SLL),In fact,small lymphoctic lymphoma and chronic lymphocytic leukemia are the virtually identical tumors. It is a disease affecting persons older than 50 years o

10、f age. Most patients are leukemic at the time of diagnosis.,24,PPT学习交流,Morphology,The lymph nodes are replaced by sheets of mature lymphocytes,which are round,small compact with dark-staining round nuclei,scanty cytoplasm and uniform in shape and size,and scattered ill-defined foci of large cells te

11、rmed prolymphocytes. The foci of mitotic active prolymphocytes are called proliferation centers,which are useful for CLL/SLL in diagnosis.,25,PPT学习交流,Immunophenotye and karyotype,The neoplastic cells express B-cell markers,such as CD19,CD20,CD23,surface immunoglobulin(e.g. IgM ,IgG).,26,PPT学习交流,27,P

12、PT学习交流,Clinical features,CLL/SLL is often asymptomatic.many cases are diagnosed as a result of routine blood tests or clinical examination for other reasons. The symptom are nonspecific,including easy fatigue,weight loss and anorexia. The prognosis is good and the patients with these diseases may su

13、rvive for 10 years or more.The median survival time is 4 to 6 years.,28,PPT学习交流,Diffuse,29,PPT学习交流,30,PPT学习交流,Follicular lymphoma,It is a tumor derived from germinal center B-cells,characterized by a nodular or follicular architecture. It is one of the commonest type of NHLs. It accounts for 10% to

14、40% of NHLs. Late adult life is a peak age incidence.,31,PPT学习交流,Immunophenotype and karyotype,CD19 ,CD20.CD10,BCL protein. Most of the cases have specific chromosome translocation involving the immunoglobulin heavy chain promoter region of chromosome 14 and the anti-apoptotic gene BCL12 on chromoso

15、me18(t14,18)(q32,q21).,32,PPT学习交流,Follicular,33,PPT学习交流,34,PPT学习交流,35,PPT学习交流,CD20,CD3,36,PPT学习交流,37,PPT学习交流,Diffuse large B-cell lymphoma,A diffuse growth pattern. Occurs mainly in older patients(median age about 60year) It is characterized by a diffuse outgrowth of large B-cells,which may display

16、centroblastic of immunblastic cytology. CD19,CD20,CD79a and IgM,38,PPT学习交流,Diffuse large cell,centroblastic,39,PPT学习交流,40,PPT学习交流,CB,IB,T/HR,ALC,CD30,CD20,CD3,41,PPT学习交流,Burkitt lymphoma,A distinctive type of B cell lymphoma. It is dndemic in para-Africa and occur much less commonly in other regions

17、.It mainly affect children and adolescents. It is associated with EBV infection and malaria. It express CD10,CD19.,42,PPT学习交流,Burkitts lymphoma,starry-sky,43,PPT学习交流,Burkitts lymphoma,mitoses,44,PPT学习交流,45,PPT学习交流,46,PPT学习交流,Peripheral T-cell lymphoma,It is relatively common in Asia compared with Am

18、erica and Europe. It is associated with human T-cell leukemia/lymphoma virus(HTL1) The morphology is variable in size and shapes of cells. The same features such as the architectures of lymph node replaced by the tumor cells. CD2,CD3,CD5,47,PPT学习交流,Lymphoblastic(T),convoluted,48,PPT学习交流,Hodgkins lym

19、phoma,Hodgkins lymphoma(also called Hodgkins disease) is a primary malignant tumor of lymphoid tissues. It is characterized by the presence of Reed-Sternberg(RS)cells in the involved tissues. It accounts for 15% of all lymphomas and shows a peak age incidence in the third and fourth decades.,49,PPT学习交流,The reasons it separated from NHLs,1 Morphologically characterized by the presence of distinctive R-S cells admixed with a variable infiltrate of reactive,nonmalignant inflammatory cells. 2 It is oft

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