TREATMENT OF REFRACTORY EVANS-SYNDROME WITH ALTERNATE-DAY CYCLOSPORINE AND PREDNISONE

Purpose: We report that the use of alternate-day cyclosporine and pred nisone improved the clinical course of a 6-year-old child with severe Evans syndrome. Before the use of cyclosporine the child had experienc ed life-threatening episodes of hemolytic anemia despite the use of mu ltiple therapeutic modalities. Methods: Cyclosporine was given at a do se of 10 mg/kg/day divided into two doses on alternate days. Results: The use of cyclosporine resulted in increased hemoglobin levels, incre ased platelet counts, and the reduction of the patient's prednisone do se from 2 mg/kg/day to as low as 1 mg/kg every other day. With this re gimen, the patient had less severe hemolytic anemia, was less thromboc ytopenic, and had fewer hospitalizations. No major toxic effects were associated with cyclosporine therapy. Conclusion: The regimen of alter nate-day cyclosporine and prednisone may prove to be useful in the tre atment of other patients with refractory Evans syndrome.