The Suppression of Haemoglobin E Synthesis When tIaemoglobin II Disease and Itaemoglobin E Trait occur together

Abstract

The blood of two infants with Haemoglobin E trait and a form of α-thalassaemia (Haemoglobin H Disease) was examined and it was confirmed that the proportion of Haemoglobin A:E was higher than in uncomplicated Haemoglobin E trait.

Haemoglobin H (β 4A) was added to the haemoglobin solution from a Haemoglobin E trait carrier. This mixture was dissociated into its α2, β 2A and β 2E subunits, and these were then recombined. The proportion of A:E had risen to that found in vivo in Haemoglobin E trait carriers with Haemoglobin H Disease.

It is suggested that competition between βA and βE for α-chains may be an example of the mechanism by which α-thalassaemia ‘interacts’ with β-chain abnormal haemoglobins.