Thrombotic Thrombocytopenic Purpura

Abstract

Serial systematic coagulation studies were carried out in a patient with clinically typical thrombotic thrombocytopenic purpura (TTP) because of the recent suggestion again that TTP is a human equivalent of the generalized Shwartzman reaction. The clotting studies failed to reveal evidence of diffuse intravascular coagulation and, therefore, fail to support this hypothesis. Evidence in the literature for considering TTP a manifestation of the generalized Schwartzman reaction was evaluated and found wanting. Reports of TTP with data on clotting factors, reports on the use of anticoagulation and reports of pathologic studies all fail to support the hypothesis that TTP is a human equivalent of the Shwartzman reaction. The disease remitted following treatment with adrenal steroids and clinical dextran. The dextran was given to prevent the formation of multiple small thrombi by coating vascular surfaces and platelets. The further trial of this therapeutic combination in TTP is recommended.