Abstract :
Beta thalassemia is the most common single gene disorder in Pakistan with a gene frequency of 5-8% and about 8-10 million carriers in the country. It has become a worldwide clinical problem due to increasing immigration of ethnic groups with high prevalence of thalassemia. Over the past three decades, regular blood transfusions and iron chelation has dramatically improved the quality of life and transformed thalassaemia from a rapidly fatal disease in early childhood to a chronic disease compatible with prolonged life.1,4 Today life expectancy varies between 25-55 years, depending on the compliance with medical treatment. Despite increased life expectancy, complications keep arising. These relate to inadequate transfusions, transfusion-related infections, allosensitization, iron-overload related cardiac, endocrine and liver disturbances and toxicities of iron chelators.4,5 Many of these problems are strongly age dependent.
