Genetic Haemoglobin Disorders in Pakistan

Abstract

Genetic haemoglobin disorders are broadly divided into thalassaemia syndromes and abnormal haemoglobins. Thalassaemias are a heterogeneous group of inherited disorders of haemoglobin synthesis in which one or more globin chains are either not produced at all (ß^o) or are produced at a reduced rate (ß+). These are classified according to the type of globin chain(s) affected. The abnormal haemoglobins are produced in normal amount but are structurally abnormal and therefore lack the usual qualities of haemoglobin. Some abnormal haemoglobins like Hb-E, in addition to being structurally abnormal are produced in reduced amount [1].