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


OBJECTIVE
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.


METHODS
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


METHODS
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.


METHODS
No trials of splenectomy for acute splenic sequestration were found.


RESULTS
No trials of splenectomy for acute splenic sequestration were found.


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.
Owusu-Ofori
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S
Department of Medicine, Komfo Anokye Teaching Hospital, P.O.Box 1934 Ghana, Kumasi, Ghana. sowusu ofori@hotmail.com
Riddington
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C
D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000150:complications*; D001803:Blood Transfusion; D006801:Humans; D013156:Splenectomy; D013158:Splenic Diseases / Q000601:surgery / Q000628:therapy*