A novel mutation of the beta-globin gene promoter (-102 C>A) and pitfalls in family screening

Am J Hematol. 2007 Dec;82(12):1088-90. doi: 10.1002/ajh.21000.

Abstract

We describe a family with beta-thalassemia in which several pitfalls of genetic diagnoses were present. These include coherent family phenotypes with discrepancies in molecular findings because of nonpaternity, and a false beta-globin gene homozygous genotype due to a large deletion in the second locus. These findings underline the difficulties of family genetic studies and the need for tight relationship between professionals involved in laboratory studies and those in-charge of the clinical follow-up and genetic counselling. In this family, we also report a new silent beta-thalassemia mutation, -102 (C>A), in the distal CACCC box of the beta-globin gene promoter.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Family
  • Female
  • Genetic Testing
  • Globins / genetics*
  • Humans
  • Male
  • Mutation*
  • Pedigree
  • Polymorphism, Single Nucleotide*
  • Promoter Regions, Genetic*
  • Reproducibility of Results

Substances

  • Globins