Summary.Our paper describes two Sardinian families with α‐β thalassaemia interaction. In the first (family S), the propositus, whose haemoglobin pattern at birth consisted of about 25 Hb Bart's and 75 Hb F, successively developed a clinical and haematological picture typical of Cooley's anaemia. Haematological and globin chain synthesis studies together with these findings suggest that he is homozygous for β0thalassaemia and heterozygous for α thalassaemia‐1 and α thalassaemia‐2. This conclusion is further substantiated by the finding of various combination of α and β thalassemia among his family members.In the P family two twins whose haemoglobin pattern and synthesis data at birth were similar to those of the proband of family S died in the neonatal period. The mother was assumed to be a compound heterozygte for α thalassaemia‐2 and β0thalassaemia and the father for α thalassaemia‐1 and β0thalassemia. The homozygous state for β0thalassaemia in association with the α thalassaemia 1 and α thalassaemia 2 genes results in a severe clinical picture similar to that of a homozygous β0thalassaemia. The interaction between the heterozygous state for β0thalassaemia and the α thalassaemia 1 or α thalassaemia 2 genes, or the combination of both, results in a haematological picture similar to that of a
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