BACKGROUND

Sickle cell disease (SCD) is an inherited autosomal recessive blood condition and is one of the most prevalent genetic blood diseases worldwide. Acute chest syndrome (ACS) is a frequent complication of sickle cell disease, as well as a major cause of morbidity and the greatest single cause of mortality in children with SCD. Standard treatment may include intravenous hydration, oxygen as treatment for hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given.

OBJECTIVES

To assess the effectiveness of blood transfusions, simple and exchange, for treating ACS by comparing improvement in symptoms and clinical outcomes against standard care.

SEARCH STRATEGY

We searched The Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings.Most recent search: 27 March 2009.

SELECTION CRITERIA

Randomised controlled trials and quasi-randomised controlled trials comparing either simple or exchange transfusion versus standard care (no transfusion) in people with sickle cell disease suffering from acute chest syndrome.

DATA COLLECTION AND ANALYSIS

No studies were identified for inclusion in the review.

MAIN RESULTS

No studies were identified for inclusion in the review.

AUTHORS' CONCLUSIONS: There is currently no reliable evidence to support or refute the effectiveness of blood transfusions as treatment options for acute chest syndrome in people with sickle cell disease. Well-designed, adequately-powered randomised controlled trials are now required to assess the benefits and risks of this form of treatment.