Beta(S) Haplotypes, alpha-globin gene status, and hematological data of sickle cell disease patients in guadeloupe (FWI) - INRAE - Institut national de recherche pour l’agriculture, l’alimentation et l’environnement Accéder directement au contenu
Article Dans Une Revue Hemoglobin Année : 1996

Beta(S) Haplotypes, alpha-globin gene status, and hematological data of sickle cell disease patients in guadeloupe (FWI)

Lisiane Kéclard
  • Fonction : Auteur
Vincent Ollendorff
Camille Berchel
  • Fonction : Auteur
Henri Loret
  • Fonction : Auteur
Guy Mérault
  • Fonction : Auteur

Résumé

The beta gene cluster haplotypes, alpha gene status, Hb F level and hematological parameters have been characterized in 154 unrelated Guadeloupe patients with sickle cell disease: 112 with sickle cell anemia, 26 with SC disease, 15 with Hb S-beta-thalassemia, and one patient with Hb S in association with the hereditary persistence of fetal hemoglobin. Fourteen haplotypes in 16 combinations were found, the three major African haplotypes were present on 92% of all chromosomes: 73% Benin, 11% Bantu, 8% Senegal. Among SS patients, 57% were Benin homozygotes, one patient was a Senegal homozygote, one patient was a Bantu homozygote, and all the others were heterozygous. The A gamma T chain was observed on seven chromosomes and about 5% of the analyzed beta S chromosomes exhibited atypical haplotypes. The common haplotype beta C was found in all patients with SC disease. An interesting feature was the high frequency (44%) of deletional alpha-thalassemia among SS patients. Two patients have an alpha-gene globin triplication. The DNA haplotypes and alpha-gene status have been correlated with hematological parameters in these patients. The anthropological aspect of these data is interesting as the haplotypes of the beta-globin gene throw light on the slave trade from the various parts of Africa to the Caribbean Islands in particular, and North America in general.
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hal-02696462 , version 1 (01-06-2020)

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Citer

Lisiane Kéclard, Vincent Ollendorff, Camille Berchel, Henri Loret, Guy Mérault. Beta(S) Haplotypes, alpha-globin gene status, and hematological data of sickle cell disease patients in guadeloupe (FWI). Hemoglobin, 1996, 20 (1), pp.63-74. ⟨10.3109/03630269609027911⟩. ⟨hal-02696462⟩

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