Abstract
Haemoglobin E (HbE) affects at least 1 million people around the world. The carrier frequency of HbE/beta-thalassaemia (HbE/β-thalassaemia) is highest in Southeast Asia. In India, the highest frequency is observed in the northeast region. Distinguishing between homozygous HbE disease and HbE/β-thalassaemia is a challenge to the haematopathologist as well as to the treating obstetrician because both are clinically and haematologically similar, posing a difficulty in managing anaemia and assessing the fetal risk for the same disease. This article reports a case of compound heterozygote HbE/β-thalassaemia in pregnancy and its successful outcome.