Abstract

The population of the United Arab Emirates (UAE) is diverse, less than 20% are UAE nationals. Within this small group a variety of single gene defects occur, of which hereditary anemia, especially beta [?]-thalassemia major; disorder of globin gene expression is one of the most prevalent. This is a transfusion-dependent severe anemia requiring lifelong blood transfusions to maintain life. It is also a major health problem in United Arab Emirates. Because of consanguinity, there are many homozygous thalassemia subjects who need special follow up and present a real health problem for their families and the community as a whole. A 5-year retrospective study from (2007-2011) of transfusion practices in hereditary anemia patients receiving multiple blood transfusions were done by analyzing the records from files of patients, available in blood bank of SAQR Hospital in Ras Al Khaimah. Our study comprised;102 patients with hereditary anemia requiring blood transfusion, with the majority of cases being ?-thalassemia major (60cases) The age range of patients was 2-23 years, with mean being 10years. It has been found more males (66%) being affected and consanguinity was found in 25% of parents of these patients and the major proportion of patients being Muslims and UAE nationals (62cases). There was a linear relationship between the age of thalassemia major patients and total number of blood transfusions received and HCV positivity. Such a relation is expected, as due to worsening of the disease with progression of age.

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