Thrombotic thrombocytopenic purpura, the acute form of which is usually fatal, is characterized clinically by thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic signs and renal dysfunction, and pathologically by diffuse thrombotic occlusions in the microcirculation, in which platelets are consumed. In the patient described in thi report, antiplatelet agents and glucocorticoids were tried but produced no clear benefit. She received repeated but transient relief from exchange transfusions and also brief improvement from splenectomy. Exchange transfusions were then shown to be effective because they provided a missing plasma factor. Infusion of large quantities of plasma effectively controlled the disease until a sustained remission ensued. It was concluded that the pathogenesis of thrombotic thrombocytopenic purpura in this patient involved a deficiency of plasma factor.
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