ABSTRACT

Reduced or absent production of ß-globin chains causes an excess of instable ß chains which are responsible for decreased erythrocyte survival, severe anemia and related complications. Anemia, iron overload and iron toxicity are the main factors in homozygous ß-thalassemia. Transfusion is very important for normal development but causes iron overload. Iron overload in thalassemic patients is variable depending on degree of anemia, degree of erythrocyte destruction, transfusional load and chelating therapy. Inappropriate iron chelation leads to progressive organ damage; the organs which are more frequently affected are the heart, liver and endocrine glands.