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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Available from: Cassandra D Josephson, Feb 10, 2015
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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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    ABSTRACT: Patients with sickle cell disease (SCD) can present several severe symptoms during their lifetime, including painful events due to vascular occlusion (VOC). Even though multiple factors are involved in VOC, hypoxia is the most important triggering factor. Inositol hexaphosphate (IHP) reduces the oxygen-haemoglobin affinity thus improving the oxygen release in the blood stream and in the tissues. Thus, IHP-loaded homologous red blood cells (IHP-RBCs) could be able to reduce disorders in SCD. The effectiveness of treatment was assessed in two types of SCD transgenic mice (BERK and SAD). The administration of four repeated injections of IHP-RBCs in BERK mice resulted in an improved survival rate and brain development, prevention of severe anaemia and a greatly lowered risk of VOC. After one injection of IHP-RBCs, SAD mice were subjected to acute hypoxic stress. Analysis of the lungs revealed significantly decreased mRNA levels of molecules involved in intravascular disorders. Our results showed that transfusion of homologous IHP-RBCs, by increasing the oxygen delivery, reduces SCD disorders in sickle transgenic mice.
    British Journal of Haematology 03/2012; 157(3):357-69. DOI:10.1111/j.1365-2141.2012.09077.x · 4.96 Impact Factor
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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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    ABSTRACT: In sickle cell disease, the complex scenario of vaso-occlusive crisis (VOC) typical of this disease is clearly multifactorial and not fully understood. Cell-cell and cell-cell matrix interactions mediated by adhesive molecules present on blood cells and endothelial cells (ECs) are thought to play an important role. Early studies have shown that sickle red blood cells (RBCs) are abnormally adherent to ECs and some of the molecules involved in these interactions have been identified, such as the alpha4beta1 integrin and CD36, exclusively present on stress reticulocytes, and CD47 on mature RBCs. More recently, attention focused on Lu/BCAM, the unique RBC receptor for laminin, and on ICAM-4, a red cell-specific adhesion receptor, which is a ligand for a large repertoire of integrins (alphaLbeta2, alphaMbeta2, alphaxbeta2, alphaVbeta3). The counter-receptors on ECs and the role of plasma proteins forming bridges between blood cells and ECs have been clarified in part. It has also been shown that reticulocytes from SCD patients express higher levels of alpha4beta1 integrin and CD36, and that under hydroxyurea (HU) therapy, both cell adhesion to ECs or extracellular matrix proteins and the levels of these adhesion molecules are reduced. These findings are consistent with the view that enhanced adhesion of blood cells to ECs is largely determined by the membrane expression level of adhesion molecules and could be a crucial factor for triggering or aggravating vaso-occlusion. In SCD patients, membrane expression of Lu/BCAM (and perhaps ICAM-4) is enhanced on RBCs whose adherence to laminin or ECs is also increased. Interestingly, Lu/BCAM- and ICAM-4-mediated adhesion are enhanced by the stress mediator epinephrine through a PKA-dependent pathway initiated by a rise in intracellular cAMP and leading to receptor activation by phosphorylation according to the same signaling pathway. More recently, studies based on quantitative expression analysis of adhesion molecules on RBCs and during erythroid differentiation in patients undergoing HU therapy, surprisingly revealed that Lu/BCAM level was enhanced, although alpha4beta1, CD36 and ICAM-4 (to a lower extent) levels were indeed reduced. CD47 and CD147 expression were also enhanced in HU-treated patients. Based on these findings we suggest that the signalization cascade leading to receptor activation rather than the expression level only of adhesion molecules may be the critical factor regulating cell adhesion, although both mechanisms are not mutually exclusive.
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    ABSTRACT: 1-3 for most hematologists such issues may seem far removed from everyday practice. Nevertheless, as genetic diseases are increasingly rec- ognized as a major global health problem, many hema- tologists are already dealing with a new spectrum of disease which they need to be able to identify and manage. Arguably the disorders which best represent how hematologists have faced the challenges posed by immigration hematology are the hemoglobinopathies, particularly sickle cell disease. The implications are, however, much wider than the direct challenge of adapting services to meet our patients' needs. We also have to consider the needs of the vast numbers of patients in the less privileged countries where these disorders are endemic: it is sobering to realise, for example, that for every birth of a child with sickle cell disease in Europe, there are more than 90 in Africa! Increasing prevalence of previously rare disorders in Europe also requires changes to education and train- ing, not only of hematologists, but of all physicians, medical students, paramedical staff and a wide variety of non-medical professional organizations indirectly involved in supporting patients with chronic disease. This article discusses the changing patterns of sickle cell disease prevalence and the implications for screen- ing, service provision, education and training; as well as the opportunities for forging global networks to work towards improving access to medical care for all affected individuals. Epidemiology: the changing pattern of sickle cell disease worldwide The hemoglobinopathies are the commonest, life- threatening, monogenic disorders in the world. Fairly recent estimates suggest that 7% of the world popula- tion are carriers and that 300,000-400,000 affected children are born every year. 4 The majority of these (approximately 250,000) have sickle cell disease. The highest frequency of sickle cell disease remains in trop- ical regions, particularly sub-Saharan Africa, India and the Middle East.
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