ALPHA-THALASSEMIA IN 2 MEDITERRANEAN POPULATIONS

  • 1 January 1982
    • journal article
    • research article
    • Vol. 60  (2) , 509-512
Abstract
Restriction endonuclease analysis was used to determine the incidence of .alpha.-thalassemia in 2 Mediterranean islands. In a random population sample, the gene frequence of deletion-type .alpha.-thalassemia-2 (-.alpha.) was 0.18 in Sardinians and 0.07 in Greek Cypriots. All cases were the rightward crossover type. From these frequencies and the known incidence of Hb-H disease in these populations, the frequency of the .alpha.-thalassemia-1 genotype (--) was calculated and found to be low. .beta.-thalassemia homozygotes in Sardinia had a higher incidence of .alpha.-thalassemia than did normals and .beta.-thalassemia heterozygotes because a significantly greater number of these homozygotes were also homozygous for the .alpha.-thalassemia-2 lesion. Coinheritance of .alpha.-thalassemia apparently mitigates the severity of .beta.-thalassemia and the protection is apparently most pronounced when 2 .alpha.-globin genes are deleted.

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