Treatment of thrombotic thrombocytopenic purpura with plasma exchange: five cases

Five patients with thrombotic thrombocytopenic purpura (TTP) were treated with corticosteroids, plasma exchange (PE), and antiplatelet agents. ABO- and Rh-compatible fresh-frozen plasma (FFP) was used as the replacement fluid. None of the patients received FFP infusion without PE. The patients were followed for 14-32 months after initial treatment. Four of the five patients are in complete clinical remission. One responded to plasma exchange but died of heart disease. This report shows an 80% survival rate which is markedly better than that without the use of PE and FFP infusion. We found dramatic clinical and hematologic improvement beginning shortly after initiating treatment. Two patients showed reversal of their initial clinical and hematologic improvement when PE was discontinued, despite continuing treatment with antiplatelet drugs and in one with corticosteroids. Both patients showed hematologic and clinical remission after reinstitution of PE. None of the survivors had any neurologic or renal deficit. They were maintained on aspirin and dipyridamole for 1 year after discharge.