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Neoplasitc Proliferative Disorders of Heme and Lymph

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Common associations with Acute Leukemias   Blasts in the bone marrow and peripheral blood Children and >60 Short path marked by anemia, infection, hemorrhage and death in 6 months  
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ALL   CHILDREN lymphoblasts in blood and marrow responsible to Tx CD10 Thrombocytopenic bleeding  
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AML   adults myeloblasts and early promyelocytes responds more poorly than ALL AUER RODS  
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Chronic Leukemias   more mature cells than acute leukemias longer and better course less responsive to Tx  
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CLL   neoplastic lymphoid cells - B-cells No plasma cells - cannot differentiate >60 yo B-cells CD19, CD20, also CD5+ and CD10- SMUDGE CELLS peripheral WBC 50-200 Complications: Warm antibody autoimmune hemolytic anemia, hypogammaglobulinemia and infectio  
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Hairy Cell Leukemia   Hairy projections TRAP + or tartarate-resistant acid phosphatase  
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CML   35-50 year olds Philadelphia chromosome t(9;22) Bcr-abl codes for tyrosine kinase protein leukocytosis WBC 50-200 mid-late myeloid precursor - segmented forms reduced leukocyte alkaline phosphatase (LAP) score  
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General features of Myeloproliferative disorders   includes p.vera and chronic idiopathic myelofibrosis Janus2 and JAK2V617F positive proliferation of myeloid cells, increased serum uric acid  
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Polycythemia Vera - abnormal serum RBC levels with decreased EPO and normal O2   erythrocytosis increasing granulocytes and platelets splenomegaly thrombotic or hemorrhagic phenomena decreased EPO pruritis  
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Secondary causes of polycythemia   chronic hypoxia from pulm diseases, high altitiude, heavy smoking inappropriate production of EPO from renal, hepatocellular, or cerebellar endocrine abnormalities like a pheochromocytoma ro Cushings  
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Chronic Idiopathic hyelofibrosis or agnogenic myeloid metaplasia and myelofibrosis with myeloid metaplasia   extramedullary hematopoesis because the marrow has become fibrotic TEAR DROP CELLS anemia splenomegaly  
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essential thrombocytopenia   thrombocytosis > 1,000,000 megakaryocytosis bleeding and thrombosis  
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Infectious Mononucleosis   non-neoplastic lymphoid proliferation EBV is cause B-cell affinity Young adults atypical lymphocytes (CD8+ T cells) marked by antibodie: anti-EBV, heterophil (sheep erythrocyte attackers) Sx: sore throat, fever, general lymphadenopathy spleen can r  
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Multiple Myeloma   plasma cell disorder - 40-50 yo lytic bone lesions punched out lesions OAF secreted by plasma cells bone pain M protein - IgG or IgA of kappa or lambda Hyperglobulinemia Bence Jones proteins rouleaux infections increase Hyper Ca amyloid myelom  
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Waldenstroms macroglobulinemia   M spike - IgM Bence Jones proteins no bone lesions plasmacytoid lymphocytes men > 50 hyperviscocity from IgM retinal vascular dilation  
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MGUS   M protein less than 2g/100mL no Bence Jones proteins less than 5% plasma proteins  
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Hodgkin lymphoma   malignant Young adults B-symptoms Reed-Sternberg Lymphocyte depletion = many RS cells = bad Lymphocyte rich = EBV Lymphocyte predominance = good prognosis, few RS cells Nodular Sclerosing - women, fibrous bands, lacunar cells, good prognosis  
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Small Lymphocytic Lymphoma   B-cell lymphoma indolent course older effacement of lymph node architecture widespread nodal involvement CLL - CD19, CD20 with CD5+ and CD10-  
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Follicular Lymphoma   B-cell indolent older MOST COMMON angulated grooved cells nodular lymph t(14;18) bcl-2  
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Mantle cell lymphoma   t(11;14)  
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Extranodal marginal zone B-cell lymphoma of MALT type   Sjogren, Hashimoto, H. Pylori associated  
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Burkitt Lymphoma   B-cell EBV starry sky macrophages are stars t(8;14) c-myc  
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cutaneous T-cell lymphomas   1) mycosis fungoides - erythema, eczema, psoriasis, CD4+ with cerebriform nucs in epidermis 2) sezary syndrome - skin lesions and cerebriform nuclei  
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Created by: burkill
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