Occurrence Of Haemoglobin H + Haemoglobin Barts In Alpha-Thalassaemia - Family With 2 Possible Homozygous Cases + With G-6-Pd-Deficiency

IT has now generally been accepted that haemoglobin H, which is a tetramer of normal β-chains, is found only in α-thalassaemia, where the deficiency of α-chains has led to a surplus of β-chains. The problem, however, why in only a small portion of cases of α-thalassaemia can Hb H be detected, has not been solved until now1. Fessas2 reports that at birth all patients with α-thalassaemia have 5 per cent or more Hb Bart's, which is a γ-chain tetramer, but that these patients afterwards develop Hb H only rarely. In family studies of patients with Hb H disease often one parent shows a thalassaemic blood picture, but no Hb H.