A case of homozygous βδ -thalassaemia is presented with review of the literature. Homozygous βδ -thalassaemia is a haemoglobinopathy where only haemoglobin F is present, haemoglobins A and A2 being totally absent. Patients with this kind of anomaly usually present with anaemia of intermediate severity. Our case, however, initially, presented with severe anaemia associated with jaundice and splenomegaly, following an episode of upper respiratory infection. Anaemia subsequently improved without any specific treatment. A pedigree study was also performed.

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