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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    ABSTRACT: Sickle cell disease (SCD) predominates in sub-Saharan Africa, East Mediterranean areas, Middle East, and India. Nigeria, being the most populous black nation in the world, bears its greatest burden in sub-Saharan Africa. The last few decades have witnessed remarkable scientific progress in the understanding of the complex pathophysiology of the disease. Improved clinical insights have heralded development and establishment of disease modifying interventions such as chronic blood transfusions, hydroxyurea therapy, and haemopoietic stem cell transplantation. Coupled with parallel improvements in general supportive, symptomatic, and preventive measures, current evidence reveals remarkable appreciation in quality of life among affected individuals in developed nations. Currently, in Nigeria and other West African states, treatment and control of SCD are largely suboptimal. Improved knowledge regarding SCD phenotypes and its comprehensive care among Nigerian physicians will enhance quality of care for affected persons. This paper therefore provides a review on the aetiopathogenesis, clinical manifestations, and management of SCD in Nigeria, with a focus on its local patterns and peculiarities. Established treatment guidelines as appropriate in the Nigerian setting are proffered, as well as recommendations for improving care of affected persons.
    Anemia 01/2015; 2015:791498. DOI:10.1155/2015/791498
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    ABSTRACT: Objective: Alloimmunization is a recognized complication of red blood cell (RBC) transfusion and causes delayed hemolytic transfusion reactions and provides problems sourcing compatible blood for future transfusions. The objective of this study was to determine the frequency of RBC alloimmunization in SCD patients in Tanzania where pretransfusion screening for alloantibodies is not practiced. Methods: In a cross-sectional study, SCD patients at Muhimbili Hospital Sickle Cell Clinic, Dar es Salaam, Tanzania, were investigated. The demographic characteristics and transfusion history were recorded. Blood samples were drawn from consenting, previously transfused patients and RBC alloimmunization was demonstrated using immunohematologic techniques. Results: There were 365 patients (median age, 16 years; 55.3% female) and they had received a median of 2 transfusion episodes. Fifteen patients (4.1%) possessed RBC alloantibodies. A total of 61 alloantibodies was found; 16 (26.2%) and 11 (18.0%), were directed against Kell and Rh blood group antigens, respectively. Conclusion: The rate of RBC alloimmunization in Tanzanian SCD patients was 4.1%. The low transfusion load may explain this immunization frequency. Nevertheless, our study confirms the significance of RBC alloimmunization as a complication in Tanzanian SCD patients. Therefore, there is need to improve immunohematologic testing in Tanzania so that RBC alloimmunization and its consequences may be prevented.
    East African journal of public health 06/2014; 11(2):775.
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