Haemoglobin E-beta-thalassaemia; a study of eight cases in Northwest India

Eight cases of haemoglobin E-beta.thalassaemia are described. Seven of them are natives of Punjab, a Northwest province of India, and one is a native of Rajasthan, a province adjacent to Punjab. Hb E is rarely encountered in Punjab, but beta-thalassaemia is relatively common. The interaction of Hb E with beta-thalassaemia resulted in a varying severity of phenotypic manifestations. An extraordinary feature in 3 out of 8 cases was the presence of a high percentage of Hb E (94 to 96%) and proportionately low Hb F (4 to 6%). The interacting thalassaemia in 5 patients was of beta0 type and in 3 patients of beta+ type.