Splenectomy: Does it still play a role in the management of thrombotic thrombocytopenic purpura?

Background: Plasma exchange is first-line therapy for patients with thrombotic thrombocytopenic purpura (TTP). Splenectomy is often indicated for patients with relapsing or refractory disease. Concerns exist about its efficacy and safety in these patients. We describe a series of patients whose TTP was treated with laparoscopic splenectomy. We also reviewed the literature in order to describe the use and safety of splenectomy for refractory or relapsing TTP. Methods: We reviewed the charts of consecutive patients with TTP referred for splenectomy and searched MEDLINE for studies describing outcomes following splenectomy for relapsing or refractory TTP. Results: In all, 5 patients were referred for relapsing TTP and underwent uneventful laparoscopic splenectomy. All 5 were in remission after more than 40 months of follow-up. We found 18 studies (87 patients) reporting the results of splenectomy for relapsing TTP and 15 studies (74 patients) involving patients who underwent splenectomy for refractory TTP. The aggregate complication (6% v. 10%) and mortality rates (1.2% v. 5%) were lower for patients who received treatment for relapsing versus refractory TTP. The rate of postsplenectomy relapse among patients with relapsing disease was 17%, whereas the nonresponse rate was 8% for patients with refractory TTP. There were no complications among the 22 laparoscopic cases reported. Conclusion: Although the data supporting splenectomy for treatment of TTP are limited to case series with no control groups, they suggest that splenectomy is an option for patients with refractory or relapsing disease. When performed laparoscopically in patients with relapsing disease, splenectomy is associated with minimal morbidity and mortality.