Excellent response of refractory life-threatening thrombotic thrombocytopenic purpura to cyclosporine treatment. Itala M, Remes K. Clinical and Laboratory Haematology 2004;26(1):65-67.

The introduction of plasma exchange (PE) has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP), and survival has increased from 10% to 80-90%. TTP refractory to PE therapy, however, is still a therapeutic challenge. This paper describes an adult patient who was initially healthy other than having insulin-dependent diabetes. She presented with nausea, vomiting, and headache with worsening confusion. She was diagnosed on day 3 of her admission with TTP based on symptoms and laboratory tests, and PE was commenced. She partially responded to multiple plasma exchanges, but remained totally dependent on plasma infusions. Several attempts to discontinue plasma therapy repeatedly lead to relapses. The TTP did not respond to vincristine, either. After three months of treatment with plasma therapy, cyclosporine was started. Plasma therapy could be discontinued after three weeks on cyclosporine, and serum LDH and blood platelet count were gradually normalized during the following two months. Cyclosporine was tapered off after six months of treatment, and the patient remained in remission. The authors conclude that cyclosporine is a worthwhile treatment option in patients with refractory TTP.