ABSTRACT

Beta thalassemia is caused by a large number of various genetic mutations or deletions of the

globin gene which cause a reduction in the synthesis and accumulation of the beta-globin

polypeptide (1-3). This gives rise to an imbalance of the beta and alpha globin chains, which in

turn, leads to the formation of significant amounts of unstable alpha globin tetramers (4-6).

These unstable tetramers give rise to oxidative damage of the red cell membrane and a decrease

in its lifespan. Thalassemias affect the populations of Mediterranean and South Asian Ancestry.

Homozygous beta thalassemia represents the most frequent form of thalassemia in the US with

an estimatd total number of 1,000 patients (7,8).