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Myeloproliferative Lymphoproliferative & Immunoproliferative disorders
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Myeloproliferative Disorders (MPD) Definition Hypercellular marrow with increased quantities of one or more cellular lineages (RBC, WBC, Plt). Features predominantly middle-aged or over insidious onset, often asymptomatic extramedullary hematopoiesis, primarily in the spleen BM fibroblastic proliferation hemorrhagic and thrombotic complications
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Examples of MPD Chronic Myelogenous Leukemia (CML) Polycythemia Vera (PV) Essential Thrombocytosis (ET) Agnogenic Myeloid Metaplasia (AMM)
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Lymphoproliferative Disorders Definition: Malignant proliferation and accumulation in BM and PB of relatively mature cells of the lymphoid lineage. Diseases Chronic Lymphocytic Leukemia (CLL) Prolymphocytic Leukemia (PLL) Hairy Cell Leukemia (HCL) Sezary Syndrome
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Other Leukemias Plasma cell leukemia: patients with near end-stage multiple myeloma Hairy cell leukemia Sezary syndrome: malignant T lymph lymphoma with circulating phase of mycosis fungoides. Skin manifestations seen
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Immunoproliferative Disorders There is a spectrum of conditions from benign to malignant involving immunoglobulin producing lymphocytic and plasmacytic cells (B cells) Monoclonal when they are produced by cells derived from a single clone: eg.M M Polyclonal :as in the normal antibody reaction to infection (a broad spectrum of globulins)
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MONOCLONAL GAMMOPATHIES ( PARAPROTEINAEMIAS ) Commonest are the types of myelomatosis IgM type is referred to as macroglobulinaemia
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Plasma Cell Dyscrasias Polyclonal gammopathy: increase in different types of immunoglobulins Monoclonal gammopathy: production of a single type of immunoglobulin. Bence Jones protein present in urine rouleaux seen on peripheral blood smear Hyperviscosity
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Multiple Myeloma
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Waldenstrom’s Macroglobulinemia plasmacytoid lymphocytes excessive IgM ESR increased plasmapheresis relieves symptoms More lymphoma-like
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