Sickle cell traits in Canada. Trimodal distribution of Hb S as a result of interaction with alpha-thalassaemia gene

Acta Haematol. 1981;65(3):157-63. doi: 10.1159/000207172.

Abstract

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 alpha-thalassaemia. Double heterozygotes for the Hb S and alpha-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.

MeSH terms

  • Adult
  • Anemia, Sickle Cell / genetics*
  • Canada
  • Child
  • Female
  • Fetal Hemoglobin / analysis
  • Hemoglobin A / analysis
  • Hemoglobin A / genetics*
  • Hemoglobin A2 / analysis
  • Hemoglobin A2 / genetics*
  • Hemoglobin, Sickle / analysis*
  • Heterozygote
  • Humans
  • Male
  • Sickle Cell Trait / blood
  • Sickle Cell Trait / genetics*
  • Thalassemia / blood
  • Thalassemia / genetics*

Substances

  • Hemoglobin, Sickle
  • Hemoglobin A
  • Hemoglobin A2
  • Fetal Hemoglobin