Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease

Abstract

BACKGROUND: Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long term management used in different parts of the world. OBJECTIVES: To assess whether splenectomy (total or partial) to prevent acute splenic sequestration crises in people with sickle cell disease improved survival and decreased morbidity in patients with sickle cell disease, as compared with regular blood transfusion. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references identified from comprehensive electronic database searches, hand searching relevant journals and hand searching abstract books of conference proceedings Additional randomized controlled trials were sought from the reference lists of the studies found and reviews identified by the search strategy. Date of the most recent search: December 2001 SELECTION CRITERIA: All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises to no treatment or blood transfusion in people with sickle cell disease. DATA COLLECTION AND ANALYSIS: No trials of splenectomy for acute splenic sequestration were found. MAIN RESULTS: No trials of splenectomy for acute splenic sequestration were found. REVIEWER'S CONCLUSIONS: Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises but there is lack of evidence from trials that it improves survival and decreases morbidity in sickle cell disease. There is a need for a well-designed, adequately powered randomised controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes as a means of improving survival and decreasing mortality from ASS in people with sickle cell disease