Effects of Alloimmunization in Sickle Cell Disease Patients

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Sickle cell disease (SCD) is a genetic disorder, characterized by defected hemoglobin (Hb) synthesis with production of an abnormal form, known as sickle hemoglobin (HbS). Under low oxygen concentration or increased body oxygen demand, red blood cells that are sickle and rigid shaped fail to pass smoothly through capillaries, with multiple clotting and thrombosis. Eventually, this leads to frequent hemolytic crisis and progressive organ damage. Transfusion is the key therapy in SCD. However, transfusion has been associated with many side effects in SCD patients, mainly iron overload and alloimmunization that cause severe hemolytic transfusion reactions. In this review, the effect of alloimmunization in transfusion practice of SCD patients will be discussed.
Blood transfusion remains a cornerstone of the management of sickle cell disease (SCD). Increasing oxygen level in tissues, and replacing sickled red blood cells by normal RBCs are two main goals for blood transfusion. However, transfusion in SCD can lead to red blood cells alloimunization with severe complications. These antibodies are often directed against antigens expressed on red blood cells of white persons, which represent the majority of donors in Western countries. Finding compatible units lacking those antigens can be difficult, time-consuming for identifying the antibodies and resulted in transfusion delays. The most serious consequence of alloimmunization is the high risk of developing delayed hemolytic transfusion reactions (DHTR), because of polymorphism differences in immunogenic blood group antigens between donors of European descent and patients of African ancestry. In these situations, the rate of alloimmunization is about 20-50%. Indeed, the i...

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...ions. Therefore, improved transfusion safety in SCD patients require both promoting donation by donors of African ancestry and ongoing research into prevention of alloimmunization and post-transfusion hemolysis.

Works Cited

1-Pirenne. F and Tournamille. C. Relevance of RH variants in transfusion of sickle cell patients. Transfusion Clinique et Biologique (2011); (18): 527-535.
2-Pirenne. F. Relevance of blood groups in transfusion of sickle cell disease patients. C.R. Biologies (2013); (336): 152-158.
3-Pirenne. F. Relevance of alloimmunization in hemolytic transfusion reaction in sickle cell disease. Transfusion Clinique et Biologique (2012); (19): 132-138.
4- Yazdanbakhsh. K, Ware. R, et al. Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. Blood Journal (2012); 120(3): 528-537.
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