Myeloproliferative Disorders (MPD) by N7vJp41

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									  Myeloproliferative
Lymphoproliferative &
 Immunoproliferative
     disorders
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
            Examples of MPD
   Chronic Myelogenous Leukemia (CML)
   Polycythemia Vera (PV)
   Essential Thrombocytosis (ET)
   Agnogenic Myeloid Metaplasia (AMM)
    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
         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
       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)
     MONOCLONAL GAMMOPATHIES
       ( PARAPROTEINAEMIAS )

   Commonest are the types of myelomatosis
   IgM type is referred to as macroglobulinaemia
           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
Multiple Myeloma
Waldenstrom’s Macroglobulinemia
   plasmacytoid lymphocytes
   excessive IgM
   ESR increased
   plasmapheresis relieves symptoms
   More lymphoma-like

								
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