Abstract

Evans syndrome is a rare, chronic, sometimes fatal immunologic disorder defined as Coombs' positive hemolytic anemia and immune thrombocytopenia without an underlying etiology. This syndrome has a variable clinical course, and the benefit of splenectomy is unknown. This report reviews the clinical outcome of laparoscopic splenectomy for Evans syndrome. A retrospective review was conducted of patients undergoing laparoscopic splenectomy for autoimmune hemolytic anemia and concomitant immune thrombocytopenic purpura at the Cleveland Clinic Foundation from August 1995 through August 2001. Data were collected in a prospective database and included demographic characteristics of the patients, surgical indications, operative details, and postoperative follow-up information. Five patients underwent laparoscopic splenectomy for Evans syndrome. At mean follow-up of 18 months (range, 1-31) after laparoscopic splenectomy, two patients had normal platelet counts (>100,000/microL) and required no further medical therapy. Two patients did not respond to laparoscopic splenectomy and are still undergoing medical therapy. One patient initially responded to laparoscopic splenectomy but became thrombocytopenic at 18 months and required further medical therapy. After 2 months of further medical treatment, this patient is currently (at month 10) in remission. Laparoscopic splenectomy for Evans syndrome is safe and technically feasible. The clinical outcome of splenectomy for patients with Evans syndrome is unpredictable. This select cohort of patients requires extensive long-term follow-up to determine the merits of splenectomy.