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1、The Diseases of Hematopoietic and lymphoid systems1荟萃医学Structure and functionHematopoietic 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 tissue
2、sThe remaining organs are actively involved in the immune response,and constitute the peripheral lymphoid tissue.2荟萃医学3荟萃医学4荟萃医学5荟萃医学6荟萃医学DiseasesInfection and reactive proliferationsThe lymphoid neoplasmasMyeloid neoplasmsHistocytic neoplasms7荟萃医学Lymphoid neoplasmsAll lymphoid neoplasms are derived
3、 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 features of tumor cells.NHLs account for about 70% t
4、o 80& of all malignant lymphomas in our country.8荟萃医学Classification of lymphoid neoplasmsThe 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)
5、 in table 9-1(page 276).9荟萃医学1948 Willis:“Nowhere in pathology has the chaos of names so clouded clear concepts as in the subject of lymphoid tumors”分类：分类： NHL分类方法多，分型繁杂分类方法多，分型繁杂10荟萃医学11荟萃医学12荟萃医学ClassificationThree major categories:1 Tumors of B-cells2 Tumors of T-cells and NK-cells3 Hodgkins lymp
6、homa13荟萃医学Non-Hodgkins lymphoma14荟萃医学Etiology(1)1.virus Murine、feline、 avian leukemias retroviruses adult T cell lymphoma-leukemia HTLV-1 Burkitts lymphoma EBV15荟萃医学Etiology(2)2.oncogenes Burkitts lymphoma: t(8;14) myc-Ig follicular lymphoma: t(14;18) bcl-2-Ig CLL:Philadelphia chromosome t(9,22) c-a
7、bl,bcr16荟萃医学Etiology(3)3.Autoimmune& immunodeficieny Disease eg. Receptor of transplanted organs. AIDS patients17荟萃医学lymphoma18荟萃医学Leukemia / LymphomaThese 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 pre
8、dominantly in children,accounting for 80% of childhood leukemia.The pre-B-cell lymphoma mainly affects children,but the Pre-T-cell tumors mainly affect adolescent males.19荟萃医学MorphologyMircoscopically,the lymph nodes affected by neoplastic cells are replaced by small to medium-sized blastcells with
9、scant cytoplasm and inconspicuous nucleoli.In blood smear slide,the nuclei of lymphoblasts with Wright-Giemsa staining show somewhat coarse and clumped chromatin and one or two nucleoli.20荟萃医学Blood and bone marrow changesIn peripheral blood,the white cell count is usually increased.sometimes more th
10、an 100,000/ul.Anemia is almost present.The platelet count is ususlly depressed to less than 100,000/ul.Bone pain and tenderness result from marrow expansion with infiltration of the subperiosteum.Splenomegaly.21荟萃医学Immunophenotype and karyotype TdT present in more than 95% of casesCD19(B cell marker
11、)CD2(T cell marker)22荟萃医学Clinical featuresThe manifestations in these diseases are similar to that of AML.Anemia,hemorrhage and infection as well as related symptoms23荟萃医学Chronic lymphocytic leukemia(CLL)/small lymphocytic lymphoma(SLL)In fact,small lymphoctic lymphoma and chronic lymphocytic leukem
12、ia are the virtually identical tumors.It is a disease affecting persons older than 50 years of age.Most patients are leukemic at the time of diagnosis.24荟萃医学MorphologyThe lymph nodes are replaced by sheets of mature lymphocytes,which are round,small compact with dark-staining round nuclei,scanty cyt
13、oplasm and uniform in shape and size,and scattered ill-defined foci of large cells termed prolymphocytes.The foci of mitotic active prolymphocytes are called proliferation centers,which are useful for CLL/SLL in diagnosis.25荟萃医学Immunophenotye and karyotypeThe neoplastic cells express B-cell markers,
14、such as CD19,CD20,CD23,surface immunoglobulin(e.g. IgM ,IgG).26荟萃医学27荟萃医学Clinical featuresCLL/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.Th
15、e prognosis is good and the patients with these diseases may survive for 10 years or more.The median survival time is 4 to 6 years.28荟萃医学Diffuse29荟萃医学30荟萃医学Follicular lymphomaIt is a tumor derived from germinal center B-cells,characterized by a nodular or follicular architecture.It is one of the com
16、monest type of NHLs.It accounts for 10% to 40% of NHLs. Late adult life is a peak age incidence.31荟萃医学Immunophenotype and karyotypeCD19 ,CD20.CD10,BCL protein.Most of the cases have specific chromosome translocation involving the immunoglobulin heavy chain promoter region of chromosome 14 and the an
17、ti-apoptotic gene BCL12 on chromosome18(t14,18)(q32,q21).32荟萃医学 Follicular33荟萃医学34荟萃医学35荟萃医学CD20CD336荟萃医学37荟萃医学Diffuse large B-cell lymphomaA 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 c
18、entroblastic of immunblastic cytology.CD19,CD20,CD79a and IgM38荟萃医学Diffuse large cell,centroblastic39荟萃医学40荟萃医学CBIBT/HRALCCD30CD20CD341荟萃医学Burkitt lymphomaA distinctive type of B cell lymphoma.It is dndemic in para-Africa and occur much less commonly in other regions.It mainly affect children and ad
19、olescents.It is associated with EBV infection and malaria.It express CD10,CD19.42荟萃医学Burkitts lymphoma,starry-sky43荟萃医学Burkitts lymphoma,mitoses44荟萃医学45荟萃医学46荟萃医学Peripheral T-cell lymphomaIt is relatively common in Asia compared with America and Europe.It is associated with human T-cell leukemia/lym
20、phoma 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,CD547荟萃医学Lymphoblastic(T),convoluted48荟萃医学Hodgkins lymphomaHodgkins lymphoma(also called Hodgkins disease) is a primary malignant tumo
21、r 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荟萃医学The reasons it separated from NHLs1 Morphologically characterized by the presence of
22、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 manifestations,such as fever.3 Its stereotypic pattern of spread allows it to be treated differently than most
23、other lymphoid neoplasm.50荟萃医学Classification of Hodgkins lymphomaIt 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 lymphoma51荟萃医学Myloid neoplasmsMyloid neoplasms arise from hematopoietic stem cells.Three types:1 Acute myeloblastic leukemia2 Mylodysplasti syndromes3 Chronic myeloproliferative disorders.52荟萃医学
