Objective: To assess whether splenectomy done during hematologic remission reduces the relapse rate in patients with relapsing thrombotic thrombocytopenic purpura. Design: Consecutive case series. Setting: Tertiary care teaching hospital. Patients: 6 consecutive patients seen during a 10-year period who had had one or more relapses of thrombotic thrombocytopenic purpura. Interventions: All patients had elective splenectomy while in hematologic remission and were iol[owed after surgery for as long as 8.0 years. Measurements: At-tack rates (events per year) were calculated for each patient from time of presentation to time of splenectomy and from time of splenectomy to January 1996. Results: A total of 26 episodes of thrombotic thrombocytopenic purpura occurred over 22.3 patient-years before splenectomy. After splenectomy, 3 acute episodes occurred over 22.7 patient-years, The attack rate (+/- 1 SD) decreased from 2.3 +/- 2.0 events per year to 0.1 +/- 0.1 events per year. Conclusion: In patients who have had one or more relapses of thrombotic thrombocytopenic purpura, splenectomy done during hematologic remission reduces the frequency of acute relapse and the resulting need for medical therapy.