Stathopoulou R, Sulis E, Lehmann H
Acta Haematol. 1979;62(2):106-11. doi: 10.1159/000207549.
A man who did not produce and beta-chains did not suffer from a severe beta-thalassaemia. He was heterozygous for Hb G Philadelphia. It has been suggested that this haemoglobin variant was associated with alpha-thalassaemia and that interaction between alpha-thalassaemia and beta-thalassaemia decreased the imbalance of alpha/beta-globin biosynthesis and thereby the severity of the beta-thalassaemic disorder. Association of Hb G Philadelphia and alpha-thalassaemia in this man and his family is now demonstrated using bone marrow and reticulocytes of the propositus and one of his sons and reticulocytes only of another son.
一名不产生β链的男子未患严重的β地中海贫血。他是Hb G费城的杂合子。有人提出，这种血红蛋白变异体与α地中海贫血有关，并且α地中海贫血和β地中海贫血之间的相互作用减少了α/β珠蛋白生物合成的失衡，从而降低了β地中海贫血症的严重程度。现在，利用先证者及其一个儿子的骨髓和网织红细胞以及另一个儿子的网织红细胞，证实了该男子及其家族中Hb G费城与α地中海贫血的关联。
