The diseases of Hematopoietic and Lymphoid SystemCharacteristics•The organs and tissues are not confined to a single anatomic site.•The cells are constantly in “traffic”.•The diseases often appear as systemic involvements.Diseases of hematopoitic and lymphoid system•Red cell disorders•White cell disorders*•Bleeding disorders•Disorders that affect the spleen and thymusWhite cell disorders•Non-Neoplastic Disorders-Reactive lymphoid hyperplasia (lymphadenitis) -Reactive leukocytosis•Neoplastic Disorders-lymphomas-myeloid neoplasms-Histiocytic and dendritic-cell neoplasmsLymph nodeBenign lymphoid hyperplasiaBenign lymphoid hyperplasia•Non-specific lymphadenitis-acute non-specific lymphadenitis-chronic non-specific lymphadenitis follicular hyperplasiaparafollicular hyperplasiasinus histiocytosis•Distinctive infectionNon-specific lymphadenitisTuberculosis lymph nodeTB•Nonspecific inflammation•distinctive infection Infection Tumor •metastatic tumors •lymphoid neoplasmsLymphoproliferative disorders with unknown reasonLymphomasLymphomas•Definition•Lymphocyte differentiation •WHO Classification•Clinical manifestation and staging •Diagnosis•Genitics•Epidemiology and etiologyLymphomas-Definition •Clonal tumors of mature and immature lymphocyte including B-cells, T-cells and Natural Killer (NK) cells and so on.•Malignant tumors of immune system.•Involved organs and tissues:-Lymphoid organsand tissues-Extranodal tissues-Bone marrow-Peripheral bloodLymphomas-Lymphocyte differentiationPhenotypic and genotypic changes in differentiation of B cells and T cellsLymphomas-WHO Classification Hodgkin lymphoma, HLNon-Hodgkin lymphoma, NHL▲B-cell neoplasms▲T-cell and NK cell neoplasmsLymphomas-staging systemLymphoma-Diagnosis•Clinical manifestations •Histopathology •Immunophenotypes •GeneticsLymphoma-Diagnosis •Biopsy•Histologic classification•Immunophenotype analysis•Genetic analysis:-Ig and/or TCR Gene rearrangement analysis -Karyotype analysis-Detection of chromosome translocation or fusion genes by FISH or PCR etcSome lymphomas with distinctive geneticabnormalitiesTypes Cytogenetic finding Genetic alteration FL t(14;18)(q32;q21)bcl-2 rearranged* MCL t(11;14)(q13;32)cyclin D1BL t(8;14)(q24;q32) myc* MALTOMA t( 11;18)(q21;q21) MALT/API2trisomy 3ALCL t(2;5)(p23;35)NPM-ALKLymphoma-Epidemiology and etiology •Chromosome translocation and oncogenes •Inherited genetic factors•Viruses-Human T-cell leukemia virus-1, HTLV-1-Epstein-Barr virus, EBV-Kaposi sarcoma herpesvirus / humanherpesvirus-8, KSHV/HHV-8-Hepatitis C virus•Bacteria-Helicobacter Pylori, HP-Chlamydia psittaci; C. pneumoniae; C. trachomatis•Environment factorsHodgkin lymphomaHodgkin lymphomaHodgkin lymphoma-characteristics• A special entity of lymphoma and occupies up to 20% of all lymphomas.•Children and young adults are more commonly involved. •The tumor arises in a single node or chain of nodes spreads to the anatomically contiguous nodes.•the neoplastic cells: Reed-Sternberg (R-S) cell and its variants.Hodgkin lymphoma -Subtypes •Nodular lymphocyte predominance Hodgkin lymphoma, NLPHL•Classical Hodgkin lymphoma, CHLLymphocyte rich, LRMixed cellularity, MCLymphocyte deplete, LDNodular sclerosis, NSCHLNodular sclerosis Lucunar cellporphyry spleenProposed signals mediated “cross talk” between R-S cells and surrounding normal cells in Classical Hodgkin lymphomaNon Hodgkin LymphomasNon-Hodgkin lymphoma-characteristics•80% to 90% of all lymphomas are NHLs •Extranodal lymphoma occupies about 1/3 of all NHLs.•Histopathologic classification is more complex.•Clinical manifestations are closely related to the sites involved.• A spectrum from lymphomas to leukemias.WHO classification for tumors of lymphoid tissues -NHLB cell neoplasms•precursor B cell neoplasms•mature( peripheral ) B cell neoplasmsT and NK cell neoplasms•precursor T cell neoplasms•mature( peripheral ) T and NK cell neoplasmsNHL –Examples •Lymphoblastic leukemia/lymphoma•Chronic lymphocytic leukemia / small lymphocytic lymphoma，CLL/SLL•Follicular lymphoma, FL•Diffuse large B-cell lymphoma，DLBCL•Burkitt lymphoma, BL•Plasma cell myeloma•Extranodal NK/T-cell lymphoma, nasal typeTdT Lymphoblastic leukemia/lymphomaChronic lymphocytic leukemia / small lymphocytic lymphomaBcl-2 Follicular lymphomaDiffuse large B-cell lymphomaBurkitt lymphomaPlasma cell myelomaExtranodal NK/T-cell lymphoma, nasal typeClinicopathologic differencesbetween HL and NHLHL NHL Patient children/young adults any aged people Stage I / II III / IV Symptoms40%15% Site LN, 90% LN, 70%neck/mediastinum extranodal,30% Dissemination predictable random Extranodal uncommon common Leukemia no yes Tumor cell R-S cells lymphocytesMyeloid neoplasmsMyeloid neoplasms -DefinitionMyeloid neoplasms arise within hematopoietic stem cells and typically give rise to monoclonal proliferations that diffusely replace normal bone marrow cells.Myeloid neoplasms : Classification•Myeloproliferative neoplasms, MPN•Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1•Myelodysplastic / myeloproliferative neoplasms, MDS/MPN•Myelodysplastic syndromes, MDS•Acute myeloid leukemia ( AML)and related precursor neoplasms•Acute leukemia of ambiguous lineageAcute myeloid leukemia( AML)•AML are tumors marked by a blockage in the differentiation of early myeloid cells.•Immature myeloid cells accumulate in the marrow, replacing normal elements, and frequently circulate in the peripheral blood.AML-clinical manifestations•Anemia•Hemorrhage•Bone pain•Fatigue•Fever•Hepatosplenomegaly•LymphadenopathyAML -DiagnosisLaboratory findings: •Peripheral bloodincreased number of white cellsimmature white cells•BM aspirationimmature white cells。
