Hematology/oncology clinics of North America
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Hematol. Oncol. Clin. North Am. · Feb 1990
ReviewThrombotic thrombocytopenic purpura and related disorders.
This article provides us with background information on the disease. Clinical features, variants and classification, laboratory findings, and pathology are discussed. Knowledge of the disease's pathogenesis has increased recently and specific causes discussed are predisposing factors, triggering agents, endothelial damage, defective PGI2 bioavailability, FVIII/vWF multimeric structure abnormalities, platelet activation, and hemolytic anemia. Proposed specific therapies discussed are steroids, heparin, antiplatelet agents, prostacyclin, splenectomy, immunosuppressive agents, plasma infusion, and plasma exchange.
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Elevated platelet counts may be encountered as a reactive phenomenon secondary to a variety of systemic conditions (thrombocytosis) or may represent a primary disorder of the bone marrow (thrombocythemia). The diagnosis of essential thrombocythemia is difficult and relies on exclusion of other myeloproliferative states and nonhematologic illnesses associated with increased platelet number. The paradoxic clinical complications of hemorrhage and thrombosis, the presence of splenomegaly, and the finding of various qualitative platelet abnormalities point to existence of the neoplastic disorder. Although treatment of the symptomatic patient with platelet lowering agents or antiplatelet drugs may be indicated and effective, the role of therapy in the asymptomatic individual remains highly controversial.