Thrombotic thrombocytopenic purpura (Moschowitz's syndrome): current physiopathologic and new therapeutical view.

Introduction. - The objective of this work was to review current data about the physiopathology, clinical features, and treatment of thrombotic thromb ocytopenic purpura (Moschowitz's syndrome). Current knowledge and key points. - Thrombotic thrombocytopenic purpura is a rare disorder characterized by widespread thrombotic injuries of platelet s in the microcirculation. Ifs physiopathology has been elucidated recently . Evidence of a deficiency of Von Willebrand's factor-cleaving protease wou ld be due to either IgG antibodies in the acute form of the disease or cons titutional deficiency in the chronic form of the disease. Future prospects and projects. - Plasma exchange is the current reference t reatment. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therap y would be more specific. (C) 2000 Editions scientifiques et medicales Else vier SAS.