Thalassaemia Intermedia : A Genetic Study in 11 Patients TABLE

Beta-thalassaemia is a hereditary defect in the synthesis of beta-polypeptide chains of haemoglobin. Most patients with beta-thalassaemia can be classified, both genetically and haematologically, into those with the homozygous form of the disease, and those with the heterozygous disease or thalassaemia minor. However, in clinical practice one occasionally encounters a patient with thalassaemia of intermediate severity, namely thalassaemia intermedia. It is the purpose of this paper to confirm the previously reported genetic heterogeneity of 'thalassaemia intermedia'.