Thrombotic thrombocytopenic purpura: Indications for and results of splenectomy

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder of unknown pathophysiology. The role of splenectomy in the multimodality therapy of TTP is controversial. Materials and methods: All charts of patients with TTP at the University of Utah between 1984 and 1994 were reviewed to evaluate various treatment regimens, and specifically, the impact of splenectomy on morbidity and survival. Results: Of the 15 patients identified, 14 underwent initial treatment with plasmapheresis and steroids. Nine patients were treated with medical therapy only, 6 of whom completely recovered, while 3 patients died. Six patients failed plasmapheresis and underwent splenectomy. There were no operative complications or postoperative deaths. All surgical patients had no active disease at last follow-up. Conclusion: Plasmapheresis and steroid administration remain the first-line therapy for TTP. This series documents that splenectomy offers excellent results with minimal morbidity and mortality in patients who do not respond to or who relapse after plasmapheresis.