CHRONIC RELAPSING THROMBOTIC THROMBOCYTOPENIC PURPURA - ROLE OF THERAPY WITH CYCLOSPORINE

Relapsing thrombotic thrombocytopenic purpura (TTP) is a rare disorder with most individuals experiencing 1 to 5 relapses, We report a patie nt with 18 episodes of thrombotic thrombocytopenic purpura (TTP), the highest number of relapses thus far described. The last 11 episodes we re treated with regimens containing cyclosporine. The patient's medica l record was reviewed for pertinent clinical, laboratory, and treatmen t data. We summarized various parameters for each episode and compared characteristics of relapses treated with vs. without cyclosporine. Th e initial episode of TTP was unusual in that it failed to respond to p lasmapheresis, glucocorticoids, and fresh frozen plasma (FFP). It remi tted only following splenectomy. Episodes 2-7 responded to FFP plus pr ednisone, Episode 8 failed to respond to prednisone plus FFP but remit ted promptly with cyclosporine plus prednisone. Subsequently, 2 relaps es responded to cyclosporine alone, 2 to cyclosporine plus FFP, 4 to c yclosporine plus prednisone +/- FFP, and 2 to cyclosporine, FFP, predn isone, and plasma exchange. There was no difference in remission durat ion, or in severity or duration of relapses treated with vs. without c yclosporine. Use of cyclosporine, however, significantly decreased the requirement for prednisone and the length of maintenance therapy; thu s it is effective mainly as an adjunctive therapy for TTP. (C) 1998 Wi ley-Liss, Inc.dagger.