Alpha-thalassaemia and globin gene rearrangements in French Polynesia

Eur J Haematol. 1995 Sep;55(3):171-7. doi: 10.1111/j.1600-0609.1995.tb00246.x.

Abstract

The prevalence of alpha-thalassaemia and various globin gene rearrangements was determined in 1992 individuals living on 11 islands in French Polynesia. The gene frequencies for alpha(+)-thalassaemia (almost exclusively the -alpha 3.7III deletion form) range from 5.3% to 19.2%. Haematological indices on 177 heterozygotes and 27 homozygotes for the -alpha 3.7III variant showed considerable overlap with indices of normal individuals; although there was a broad correlation of average indices with alpha-globin genotype, individual values were a poor indication of genotype. A non-deletion form of alpha(+)-thalassaemia (alpha alpha Th), triplicated alpha genes (alpha alpha alpha) and single zeta gene (-zeta) chromosomes were present at low frequencies (< 1%), whereas triplicated gamma gene (gamma gamma gamma) and triplicated zeta (zeta zeta zeta) arrangements were more common (1.1-16.3%). alpha 0-thalassaemia, probably introduced from Southeast Asia in the early part of this century, was observed in a number of individuals of Chinese and Chinese/Polynesian ancestry. Because of the high frequency of alpha(+)-Thalassaemia on some islands, it therefore seems likely that haemoglobin H disease (resulting from the interaction between alpha 0 and alpha(+)-thalassaemia) must occur in parts of French Polynesia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Erythrocyte Indices
  • Female
  • Gene Deletion
  • Gene Frequency
  • Gene Rearrangement*
  • Globins / genetics*
  • Haplotypes
  • Heterozygote
  • Homozygote
  • Humans
  • Male
  • Middle Aged
  • Polynesia / epidemiology
  • alpha-Thalassemia / epidemiology*
  • alpha-Thalassemia / genetics

Substances

  • Globins