Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disturbances, and renal failure. Plasma therapy has dramatically improved prognosis of TTP, whereas recurrent acute episodes still occur in approximately 40% of patients. Moreover, patients with acquired ADAMTS13 deficiency, which is a significant factor for relapse, may require additional immunosuppressive treatment to get a durable remission. STUDY DESIGN AND METHODS: We hereby report two patients with a history of relapsed idiopathic TTP, who both received cyclosporin A (CSA) as a prophylactic manner after the remission was achieved. We also discuss the efficacy of CSA in patients with relapsed idiopathic TTP with a review of the published literature. RESULTS: Under CSA therapy, both patients maintained their clinical remission state, and the ADAMTS13 levels were normalized. CONCLUSION: To conclude, CSA therapy may be useful for the prevention of relapsed idiopathic TTP in patients with a history of frequent relapses.