A trimodal distribution of the amounts of Hb S in 56 sickle cell heterozygotes was observed. The variable amounts of Hb S were directly related to the mean cell volume and the mean cell Hb and were a result of the difference in net synthesis of Hb S due to the presence of the α-thalassaemia. Double heterozygotes for the Hb S and α-thalassaemia-1 genes, besides having a lower amount of Hb S in the red blood cells, were found to have lower Hb and slightly elevated Hb A2 levels.
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© 1981 S. Karger AG, Basel
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