Molecular heterogeneity of beta-thalassemia alleles in Spain and its importance in the diagnosis and prevention of beta-thalassemia major and sickle cell disorders

Hemoglobin. 2009;33(3):226-34. doi: 10.1080/03630260903089003.

Abstract

In the last 20 years, migratory flows have changed the pattern of beta-thalassemia (beta-thal) mutations in Catalonia and have also increased beta(S) prevalence, either alone or in association with beta-thal alleles. Characterization of the beta gene is needed for genetic counseling for beta-thal major and also for sickle cell diseases. The purpose of this study was to investigate the current distribution pattern of beta-thal mutations. Seventy nine individuals were characterized at the molecular level. As a first step, frequent mutations in the Mediterranean region were screened and when none of these mutations were identified, the beta-globin gene was sequenced. Screening for common mutations allowed the characterization of 60 individuals. In the remaining 19 cases, 11 different mutations were identified. beta-Thalassemia heterogeneity in Spain has markedly increased, leading to the requirement of including new methods for genetic diagnosis. Prevention of beta-thal major and sickle cell disease are necessary since their prevalence in Spain is increasing dramatically.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles*
  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / genetics*
  • Anemia, Sickle Cell / prevention & control
  • DNA Mutational Analysis
  • Gene Frequency
  • Genetic Heterogeneity
  • Genetic Testing
  • Humans
  • Mutation*
  • Spain
  • beta-Globins / genetics
  • beta-Thalassemia / diagnosis
  • beta-Thalassemia / genetics*
  • beta-Thalassemia / prevention & control

Substances

  • beta-Globins