Excellent response of refractory life-threatening thrombotic thrombocytopenic purpura to cyclosporine treatment

Abstract

The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP), and survival has increased from 10 to 80–90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained totally dependent on plasma infusions. Several attempts to discontinue plasma therapy repeatedly lead to relapses. TTP did not response to vincristine, either. After 3 months treatment with plasma therapy, cyclosporine was started. Plasma therapy could be discontinued after 3 weeks on cyclosporine, and serum LDH and blood platelet count were gradually normalized during 2 months. Cyclosporine was tapered off after 6 months treatment, and the patient has stayed in remission ever since. We conclude that cyclosporine is a worthwhile treatment option in patients with refractory TTP.