Dominantly Inherited β-Thalassemia Caused by a Single Nucleotide Deletion in Exon 3 of the β-Globin Gene: Hb Xiangyang (HBB: c.393delT)

Hemoglobin. 2022 Jul;46(4):253-255. doi: 10.1080/03630269.2022.2072325. Epub 2022 Jun 10.

Abstract

We report a de novo frameshift mutation in exon 3 of the β-globin gene that leads to a β-thalassemia (β-thal) intermedia (β-TI) phenotype in a 6-year-old Chinese boy. This novel mutation with deletion of the last nucleotide (-T) at codon 130 results in a β-globin chain that is extended to 156 amino acid residues. This study highlights the importance of considering dominantly inherited β-thal in the investigation of anemia, even in patients with ethnic backgrounds not usually associated with β-thal and hematologically normal parents.

Keywords: Abnormal hemoglobin (Hb) variant; Hb Xiangyang; dominantly inherited β-thalassemia (β-thal); inclusion bodies.

MeSH terms

  • Codon
  • Exons
  • Humans
  • Mutation
  • Nucleotides
  • beta-Globins* / chemistry
  • beta-Globins* / genetics
  • beta-Thalassemia* / diagnosis
  • beta-Thalassemia* / genetics

Substances

  • beta-Globins
  • Nucleotides
  • Codon