• Ceri Hirst and Lorna Williamson.
• AstraZeneca, Parklands (90HW2Q1), Alderley Park, Cheshire, SK10 4TG, UK. Ceri.Hirst@astrazeneca.com
• Cochrane Db Syst Rev. 2012 Jan 1;1:CD003149.

BackgroundSickle cell disease is one of the most common inherited diseases in the world, and can cause haemolytic anaemia, vaso-occlusive crises and dysfunction in virtually any organ system in the body. Surgical procedures are often required. Blood transfusion regimens can be used preoperatively in an attempt to increase transport of oxygen around the body and dilute the sickled red blood cells, thus reducing the risk of vaso-occlusion.ObjectivesTo assess the relative risks and benefits of preoperative blood transfusion regimens in people with sickle cell disease undergoing surgery of any type in any setting.Search MethodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 06 October 2011.Selection CriteriaAll randomised or quasi-randomised controlled studies comparing preoperative blood transfusion regimens to different regimens or no transfusion in people with sickle cell disease undergoing surgery.Data Collection And AnalysisBoth authors independently assessed the risk of bias of the included studies and extracted data.Main ResultsThe searches identified three studies, of which two, involving a total of 920 participants, were eligible for inclusion in the review. The first study compared an aggressive transfusion regimen (decreasing sickle haemoglobin to less than 30%) to a conservative transfusion regimen (increasing haemoglobin to 10 g/dl) in 604 elective operations in people with sickle cell disease. The conservative regimen was found to be as effective as the aggressive regimen in preventing perioperative complications, and was associated with fewer transfusion-related adverse events. The second study compared a preoperative transfusion group to a group receiving standard care, and did not show an advantage to preoperative transfusion.Authors' ConclusionsWhile in general, conservative therapy appears to be as effective as aggressive therapy in preparation for surgery in people with sickle cell disease, further research is needed to examine the optimal regimen for different surgical types, and to address whether preoperative transfusion is needed in all surgical situations.

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