Myeloproliferative Lymphoproliferative Immunoproliferative disorders Myeloproliferative Disorders MPD n

Myeloproliferative Lymphoproliferative & Immunoproliferative disorders

Myeloproliferative Disorders (MPD) n Definition q n Hypercellular marrow with increased quantities of one or more cellular lineages (RBC, WBC, Plt). Features q q q 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 n n Chronic Myelogenous Leukemia (CML) Polycythemia Vera (PV) Essential Thrombocytosis (ET) Agnogenic Myeloid Metaplasia (AMM)

Lymphoproliferative Disorders n n Definition: Malignant proliferation and accumulation in BM and PB of relatively mature cells of the lymphoid lineage. Diseases q q Chronic Lymphocytic Leukemia (CLL) Prolymphocytic Leukemia (PLL) Hairy Cell Leukemia (HCL) Sezary Syndrome

Other Leukemias n n n Plasma cell leukemia: patients with near end-stage multiple myeloma Hairy cell leukemia Sezary syndrome: malignant T lymphoma with circulating phase of mycosis fungoides. Skin manifestations seen

Immunoproliferative Disorders n n n 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 ) n n Commonest are the types of myelomatosis Ig. M type is referred to as macroglobulinaemia

Plasma Cell Dyscrasias n n Polyclonal gammopathy: increase in different types of immunoglobulins Monoclonal gammopathy: production of a single type of immunoglobulin. q q q Bence Jones protein present in urine rouleaux seen on peripheral blood smear Hyperviscosity

Multiple Myeloma

Waldenstrom’s Macroglobulinemia n n n plasmacytoid lymphocytes excessive Ig. M ESR increased plasmapheresis relieves symptoms More lymphoma-like