The Correlation between Red-Cell Survival and Excess of α-Globin Synthesis in β-Thalassaemia

In 13 subjects affected by β-thalassaemia major, in three subjects affected by β-thalassaemia minor and in five normal healthy persons haemoglobin synthesis and the survival of red cells transfused into normal, group compatible, healthy recipients has been studied. The existence of an excess of newly synthesized α-chains and of a negative correlation between the excess α-chain and the red-cell survival has been demonstrated. The harmful role of the α-chain excess on the erythrocyte and the implications of this finding are discussed.