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Thrombotic Thrombocytopenic Purpura

Johns Hopkins University Hospital, Department of Medicine, Division of Hematology, 600 N Wolfe St, Baltimore, MD 21205

William R. Bell, MD

Johns Hopkins University Hospital, Department of Medicine, Division of Hematology, 600 N Wolfe St, Baltimore, MD 21205

Thrombotic thrombocytopenic purpura (TTP) is classically highlighted by a pentad of features: fever, hemolytic anemia, thrombocytopenia purpura, transient or permanent central nervous system signs, and renal disease. The antemortem diagnosis is reliant upon the multisystem clinical signs and symptoms in conjunction with severe hemolytic anemia and thrombocytopenia. Relapse is common within the first six months after initial presentation. Laboratory findings have been generally nonspecific per se, and antemortem tissue biopsy findings are frequently unrewarding. Recently, however, unusually large multimers of the Factor VIII:Ag molecule (von Willebrand protein) have been identified in the plasma of patients with TTP who have recovered from an acute attack. This observation is very important because it may lead to essential information on the nature of the inciting event in this devastating illness. The differential diagnosis includes several multisystem disease processes such as the hemolytic uremic syndrome, toxemia of pregnancy, systemic lupus erythematosus, subacute bacterial endocarditis, nonbacterial thrombotic endocarditis, immune thrombocytopenic purpura, and the postpartum renal failure syndrome. The hemolytic uremic syndrome, toxemia of pregnancy, and TTP may resemble each other, exhibit many overlapping features, and are probably related. The cause of TTP remains unknown; the overwhelming majority of cases occur in otherwise healthy people without any recognizable underlying illness. Since 1965 45 to 70% of patients survive, a significant improvement in contrast to the early 1900s when the mortality rate was greater than 90%. The most dramatic advance has been observed in therapeutics, namely the utilization of some mode of plasma therapy (either infusion alone or plasmapheresis followed by plasma infusion). Corticosteroids remain very important in the management of patients with TTP. Vincristine may be very helpful, but additional studies are needed. The efficacy of vinca alkaloids, chronic immunosuppressive therapy, and sple. nectomy remains undefined. At present there is very little, if any, evidence that antiplatelet agents, aspirin, and prostacyclin are beneficial to patients with TTP. Prompt diagnosis and vigorous aggressive therapy is critical for successful management of TTP patients.

Journal of Intensive Care Medicine, Vol. 1, No. 6, 341-355 (1986)
DOI: 10.1177/088506668600100606


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