Management of transfusion dependent thalassaemias (TDT) in the Maldives

Abstract

ABOUT THALASSAEMIA Definition halassaemia is a group of blood disorder characterized by decreased or absent synthesis of normal globin chains. According to the chain whose synthesis is impaired, the thalassaemia is called α-, β-, γ-, δ -, δβ-, or εγδβ-thalassaemias. Thalassaemia comes under the broad spectrum of genetic blood disorders known as haemoglobinopathies. Clinical picture of haemoglobinopathies can vary from patients who require occasional blood transfusions (such as HbE-Beta thalassaemia) to patients who require regular lifelong blood transfusions (such as Beta thalassaemia major). Thalassemia is very common in Maldives. Any child present with severe anaemia should be investigated for both thalassemia and iron deficiency anaemia. Genetic basis of thalassaemia Most thalassaemia is inherited as autosomal recessive traits. The Figure 1 below gives the risk categorization of affected children from different partner matches.