Effective cyclosporine therapy to resistant-Evans syndrome

Evans syndrome is a rare disease defined as autoimmune hemolytic anemia plus immune thrombocytopenia. We describe a 57-year-old man with Evans syndrome whose thrombocytopenia was refractory to conventional therapy, including prednisolone, danazol, azathiopurine, cyclophosphamide, vincristine, gamma-globulin and splenectomy. The patient was then treated with three cycles of pulsed high-dose dexamethasone (40 mg/day for 4 sequential days every 4 weeks) followed by cyclosporine A therapy (300 mg/day). The platelet counts dramatically increased from 2,000/microliter to 200,000/microliter. Although thrombocytopenia appeared again with the tapering of cyclosporine A, the platelet counts recovered by re-increase of cyclosporine A, and have remained above 100,000/microliter for more than 14 months. Treatment with cyclosporine A can provide an effective therapeutic choice for refractory Evans syndrome.