Alpha Thalassemia and Haplotypes of Beta Globin in Sickle Cell Disease

Sickle cell disease (DF) is the most common hereditary monogenic disease in Brazil. The term DF includes sickle cell anemia (AF) and pathological conditions in which the hemoglobin S gene is associated with other hereditary hemoglobinopathies such as SC, S/beta0 and S/beta+ thalassemia (S/b), SD Punjab, among others. There is a description of 5 haplotypes of the globine chain cluster (¿S) linked to HbS: Asia, Senegal, Benin, Bantu (CAR) and Cameroon. The DF can also coexist with ¿-talassemias (¿-Tal). The most severe complication in the DF is stroke, which is responsible for 20% of mortality. The causes and factors that would increase the risk of strokes are not completely known. Several lines of evidence suggest that a genetic signature could influence the development of strokes.