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