Cyclic relapses of thrombotic thrombocytopenic purpura

Abstract

A 24-year-old male patient was first observed with full-blown acute thrombotic thrombocytopenic purpura in 1991. Complete remission was achieved with plasma and plasmapheresis therapy, but in spite of continuous corticosteroid and aspirin administration, thrombocytopenic (megakaryocytic) relapses were observed every 26–30 days. Splenectomy and danazol failed to prevent the recurrence of the disease. Surprisingly, cyclosporin A (5 mg/kg/day) administration resulted in a complete transitional remission, but after dose reduction a less regular pattern of repeated milder recurrences was observed. Cryopreserved plasma, obtained from the patient during remission also proved to be effective in treating the last two thrombocytopenic episodes.