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Effect of α-thalassemia and β-globin gene cluster haplotypes on the hematological and clinical features of sickle-cell anemia in Brazil

✍ Scribed by Figueiredo, M.S.; Kerbauy, J.; Gonçalves, M.S.; Arruda, V.R.; Saad, S.T.O.; Sonati, M.F.; Stoming, T.; Costa, F.F.

Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
447 KB
Volume
53
Category
Article
ISSN
0361-8609

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✦ Synopsis

To compare the features of sickle-cell anemia in Brazil with those in other locales, we studied the effects of the p-globin-like gene cluster haplotype and a-thalassemia upon the clinical and hematological features in 85 patients. The distribution of haplotypes differed from that in the United States and Jamaica. The Central African Republic (CAR) haplotype predominated; 34% of patients were CAR haplotype homozygotes, 45% CAW Benin homozygotes, and 11 % Benin homozygotes. No Senegal haplotype chromosomes were observed. a-thalassemia was present in 17.5% of patients. HbF levels were higher in Benin homozygotes, compared with the other two groups (P < 0.05). Nearly half the patients with a CAR haplotype had leg ulcers, compared to 12.5% of the Benin homozygote group; stroke did not occur in a-thalassemia carriers, but neither result was statistically significant. As in other studies, our results indicate that the CAR haplotype may be associated with more severe disease.

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