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

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

Autor Figueiredo, Maria Stella Autor UNIFESP Google Scholar
Kerbauy, José Autor UNIFESP Google Scholar
Goncalves, M. S. Google Scholar
Arruda, V. R. Google Scholar
Saad, STO Google Scholar
Sonati, M. F. Google Scholar
Stoming, T. Google Scholar
Costa, F. F. Google Scholar
Instituição Universidade Estadual de Campinas (UNICAMP)
Universidade Federal de São Paulo (UNIFESP)
MED COLL GEORGIA
Resumo To compare the features of sickle-cell anemia in Brazil with those in other locales, we studied the effects of the beta-globin-like gene cluster haplotype and alpha-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% CAR/Benin homozygotes, and 71% Benin homozygotes. No Senegal haplotype chromosomes were observed, alpha-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 alpha-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. (C) 1996 Wiley-Liss, Inc.
Palavra-chave sickle-cell disease
clinical features
hemoglobin S
haplotypes
alpha-thalassemia
Idioma Inglês
Data de publicação 1996-10-01
Publicado em American Journal of Hematology. New York: Wiley-liss, v. 53, n. 2, p. 72-76, 1996.
ISSN 0361-8609 (Sherpa/Romeo, fator de impacto)
Publicador Wiley-Blackwell
Extensão 72-76
Fonte http://dx.doi.org/10.1002/(SICI)1096-8652(199610)53:2<72
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:A1996VQ09900003
Endereço permanente http://repositorio.unifesp.br/handle/11600/25626

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