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 followed after surgery for as long as 8.0 years.
Measurements: Attack 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.