XXXX : indications for and results of splenectomy.

@entity1 @entity134 @entity230 @entity204 @entity2106
BACKGROUND: @entity2106 ( @entity2106 ) is a rare, life-threatening disorder of unknown pathophysiology. The role of splenectomy in the multimodality therapy of @entity2106 is controversial. MATERIALS AND METHODS: All charts of @entity1 with @entity2106 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 @entity1 identified, 14 underwent initial treatment with plasmapheresis and @entity230 . Nine @entity1 were treated with medical therapy only, 6 of whom completely recovered, while 3 @entity1 died. Six @entity1 failed plasmapheresis and underwent splenectomy. There were no operative complications or @entity204 . All surgical @entity1 had no active disease at last follow-up. CONCLUSION: Plasmapheresis and @entity134 administration remain the first-line therapy for @entity2106 . This series documents that splenectomy offers excellent results with minimal morbidity and mortality in @entity1 who do not respond to or who relapse after plasmapheresis.
