Article

Transfusion in the Patient With Sickle Cell Disease: A Critical Review of the Literature and Transfusion Guidelines

Transfusion Medicine Program, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA.
Transfusion Medicine Reviews (Impact Factor: 4.54). 04/2007; 21(2):118-33. DOI: 10.1016/j.tmrv.2006.11.003
Source: PubMed

ABSTRACT The clinical outcomes of sickle cell disease (SCD) have vastly improved over the years in great part as a result of advanced medical technologies, improved patient education, and multidisciplinary care. A key component in the successful management of patients with SCD is red blood cell transfusion therapy used in the treatment and prevention of sickle cell complications. However, although the successful application of transfusion therapy has significantly improved the morbidity and mortality of patients with SCD, the literature that addresses the appropriate selection and use of blood products continues to evolve with no clear universal standard of care. Our objectives were to provide an in-depth review of the current literature on transfusion therapy in SCD and to provide a set of guidelines for the transfusion management of patients with SCD.

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    • "Blood transfusions are currently the most studied and accepted therapy for those patients. Chronic red blood cell (RBC) exchanges are effective in preventing the recurrence of strokes or VOC (Wayne et al, 1993; Pegelow et al, 1995; Adams et al, 1998; Miller et al, 2001; Josephson et al, 2007) while simple blood transfusions are usually used in case of severe anaemia or ACS (Hirani et al, 2011). For chronic and acute transfusions, the goal is to increase the oxygen carrying capacity and to dilute abnormal SS-RBCs in whole blood. "
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    • "Therapeutic strategies targeting these different aspects of SCD have been developed [24,25,52–57]. Interestingly, the fast beneficial effect of transfusions of normal RBCs to SCD patients, relieving pain and erythrocyte sequestration, indicate that VOCs are reversible before infarction occurs [58] "
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