title: Thrombotic thrombocytopenic purpura and related disorders.
abstract: 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.
authors.affiliation: Ospedali Riuniti di Bergamo, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Ruggenenti
authors.forename: G
authors.initials: G
authors.lastname: Remuzzi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003131:Combined Modality Therapy; D003276:Contraceptives, Oral / Q000009:adverse effects; D003937:Diagnosis, Differential; D004198:Disease Susceptibility; D004730:Endothelium, Vascular / Q000473:pathology; D011464:Epoprostenol / Q000172:deficiency; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007239:Infections / Q000150:complications; D008297:Male; D008875:Middle Aged; D015539:Platelet Activation; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy
chemical_list: D003276:Contraceptives, Oral; D007166:Immunosuppressive Agents; D011464:Epoprostenol