Medical and Dental Implications of Patients with Beta Thalassaemia Major. Part1: General and Medical Characteristics: A Review

Abstract

Abstract: Thalassemia is one of the most common inherited haemoglobinopathies characterized by either a partial or a complete suppression in the production of normal haemoglobin as a result of defective synthesis of one or more of the globins chains. Thalassemia is the most widely distributed genetic disorder. Approximately 5 % of the world’s population was found to have a globin variant, with only 1.7 % having an alpha or beta thalassemia trait. The Mediterranean region, the Middle East, the Indian subcontinent and Far East Asia show the highest rates of prevalence of beta thalassemia. Beta thalassemia is considered to be a major public health issue, as well as a life threating condition characterized by severe anaemia, hepatosplenomegaly, growth retardation, endocrine dysfunction, cardiac failure and skeletal changes.

Management strategies using blood transfusion and chelation therapy will improve the control of anaemia, suppression of erythropoiesis, and inhibition of gastrointestinal iron absorption of beta thalassaemia major patients. Bone marrow transplant is considered an excellent curative therapy in thalassaemic patients during childhood, which will provide prolonging life expectancy.

This paper reviews the literatures and discusses briefly the definition, history, epidemiology, classification, aetiopathogenesis, general manifestation and general management of beta thalassaemia major.