Amal El-Beshlawy

Prof. of Pediatric Hematology Cairo University

Thalassemia is the commonest hereditary hemolytic anemia in Egypt. The carrier rate varies from 6 to 10 %, gene frequency is 0.03. It was estimated that 1000 per 1.5 million live births per year is born with thalassemia. The number of patients are steadily increasing to reach more than 2000 till 2003 in the biggest hematology center in Cairo university with 60% of the patients are less than 10 years  30% from 10-18 years and 10%  > 18 years

Now we have more than 25 centers for the patient care affiliated to universities mainly Cairo and Ain Shams, Ministry of health and the Egyptian Red Crescent. Blood transfusion, chelation therapy and others are provided to the patients free of charge. The Egyptian thalassemia association has been established in 1990 to support the patients medically and socially, it is a member of TIF since 1992.

Our patients are maintained on Hb level of 9 g/dl and chelation therapy with desferroxamine (DFO) but only 48% are compliant. Cardiac complications are the main cause of morbidity and mortality among our patients in the 2nd and 3rd decade of life. Hepatitis C is a common problem as PCR is positive in 74 % of the cases. Bone marrow transplantation (BMT) is curative in 80% of the patients (n:40). Prenatal diagnosis was carried out for some cases and genetic counseling centers are remarkably few. L-carnitine a butyrate derivative is widely used in Egypt, it improves the cardiac status, Hb levels, growth and general well being of our thalassemics. Prevention of thalassemia and establishing more BMT and thalassemia care centers are our future goals for thalassemia in Egypt