Transfusion and sickle cell anemia in Africa.

Sickle cell anemia (SCA) is the commonest life-threatening genetic disorder in tropical regions, particularly in sub-Saharan Africa. It has been estimated that between 50-90% of SCA children will die in Africa before the age of 5, corresponding to a number of 150,000-300,000 annual SCA child deaths, which represents 5-10% of total child mortality. Transfusion support remains an essential component in the management of patients with SCA and has made a significant contribution to improving patient morbidity and mortality. In Africa where the majority of patients with SCA reside, many blood transfusion challenges remains, including shortage of blood supplies, risks related to infectious and immunologic potential side effects and limitation on the diagnosis and management of post-transfusion iron overload. The proportion of transfused SCA patients varies from different studies, between 30% and 90%. This variation can be related to environmental factors, disease genetic factors and other factors including the low availability of blood, difficulties in accessing to health care and inadequacies of the transfusion system. Because blood transfusion therapy is an integral component of the management of SCA, improved efforts and strategies to overcome these challenges and optimize blood transfusion practices are needed in African countries.