1、The Diseases of Hematopoietic and lymphoid systems,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 lymphoid tissues
2、The remaining organs are actively involved in the immune response,and constitute the peripheral lymphoid tissue.,Diseases,Infection and reactive proliferations The lymphoid neoplasmas Myeloid neoplasms Histocytic neoplasms,Lymphoid neoplasms,All lymphoid neoplasms are derived from a single transform
3、ed 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 features of tumor cells. NHLs account for about 70% to 80& of all malignan
4、t lymphomas in our country.,Classification of lymphoid neoplasms,The aim of classification is to identify homogenerous subgroups that behave in a predictable way. The lymphoid neoplasms are named according to the normal cell they most closely resemble. WHO classifications(2000) in table 9-1(page 276
5、).,1948 Willis: “Nowhere in pathology has the chaos of names so clouded clear concepts as in the subject of lymphoid tumors”,分类：NHL分类方法多，分型繁杂,Classification,Three major categories: 1 Tumors of B-cells 2 Tumors of T-cells and NK-cells 3 Hodgkins lymphoma,Non-Hodgkins lymphoma,Etiology(1),1.virusMurin
6、e、feline、 avian leukemias retrovirusesadult T cell lymphoma-leukemia HTLV-1Burkitts lymphoma EBV,Etiology(2),2.oncogenesBurkitts lymphoma: t(8;14)myc-Igfollicular lymphoma: t(14;18)bcl-2-IgCLL:Philadelphia chromosome t(9,22)c-abl,bcr,Etiology(3),3.Autoimmune&immunodeficieny Diseaseeg. Receptor of tr
7、ansplanted organs.AIDS patients,lymphoma,Leukemia / 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
8、 mainly affects children,but the Pre-T-cell tumors mainly affect adolescent males.,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
9、 with Wright-Giemsa staining show somewhat coarse and clumped chromatin and one or two nucleoli.,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
10、100,000/ul. Bone pain and tenderness result from marrow expansion with infiltration of the subperiosteum. Splenomegaly.,Immunophenotype and karyotype,TdT present in more than 95% of cases CD19(B cell marker) CD2(T cell marker),Clinical features,The manifestations in these diseases are similar to tha
11、t of AML. Anemia,hemorrhage and infection as well as related symptoms,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 of age.
12、 Most patients are leukemic at the time of diagnosis.,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 termed prolymphocyt
13、es. The foci of mitotic active prolymphocytes are called proliferation centers,which are useful for CLL/SLL in diagnosis.,Immunophenotye and karyotype,The neoplastic cells express B-cell markers,such as CD19,CD20,CD23,surface immunoglobulin(e.g. IgM ,IgG).,Clinical features,CLL/SLL is often asymptom
14、atic.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 survive for 10 years or more.The median survival tim
15、e is 4 to 6 years.,Diffuse,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 40% of NHLs. Late adult life is a peak age incidence.,Immunophenotype and karyotype
16、,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 chromosome18(t14,18)(q32,q21).,Follicular,CD20,CD3,Diffuse large B-cell lymphoma,A diffuse growth patt
17、ern. Occurs mainly in older patients(median age about 60year) It is characterized by a diffuse outgrowth of large B-cells,which may display centroblastic of immunblastic cytology. CD19,CD20,CD79a and IgM,Diffuse large cell,centroblastic,CB,IB,T/HR,ALC,CD30,CD20,CD3,Burkitt lymphoma,A distinctive typ
18、e of B cell lymphoma. It is dndemic in para-Africa and occur much less commonly in other regions.It mainly affect children and adolescents. It is associated with EBV infection and malaria. It express CD10,CD19.,Burkitts lymphoma,starry-sky,Burkitts lymphoma,mitoses,Peripheral T-cell lymphoma,It is r
19、elatively common in Asia compared with America 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,Lymphoblastic(T),conv
20、oluted,Hodgkins lymphoma,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 four
21、th decades.,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 often associated with somewhat distinctive clinical features,including systemic manifestatio
22、ns,such as fever. 3 Its stereotypic pattern of spread allows it to be treated differently than most other lymphoid neoplasm.,Classification of Hodgkins lymphoma,It is classified based on the appearance of the abnormal cells and the reactive cells under the microscope. I.e. Nodular lymphocyte predominant Hodgkins lymphoma and Classic Hodgkins lymphoma,Myloid neoplasms,Myloid neoplasms arise from hematopoietic stem cells. Three types: 1 Acute myeloblastic leukemia 2 Mylodysplasti syndromes 3 Chronic myeloproliferative disorders.,
