3 cases of thalassemia intermedia have been found in the same family. The parents are not consanguineous but both come from the same town of Calabria (Italia). The mother is a heterozygote for β-thalassemia, as well as the father whose globin chain synthesis is nevertheless balanced, thus suggesting an association with α-thalassemia. This hypothesis is confirmed by the fact that one of the offspring shows the typical characteristics of α-thalassemia heterozygosity. The 3 subjects with thalassemia intermedia are synthesizing the β+-globin chain in a proportion higher than that expected from the level of Hb A in peripheral blood. In 2 of them, the globin chain biosynthetic ratio measured in the blood reticulocytes is not significantly different from that usually observed in thalassemia major of either the β° or β+ type. In the third subject the globin chain synthesis is slightly less unbalanced probably because an α-thalassemia is also present. This suggests that factors other than a lesser imbalance in globin chain synthesis are involved in the occurrence of thalassemia intermedia. One of these factors could be a better survival of cells richer in Hb F than in Hb A, since these cells must have a lesser excess of α-chains.

Keywords:
β+-Thalassemia intermedia, Globin chain synthesis
© 1980 S. Karger AG, Basel
1980
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