Identification of a Novel 7-bp Deletion in the α-Globin Gene Cluster in One Chinese Family

Hemoglobin. 2023 Nov;47(2):49-51. doi: 10.1080/03630269.2023.2216890. Epub 2023 May 29.

Abstract

Deletional α-thalassemia is characterized by reduced hemoglobin A2 and involves the deletion of a few nucleotides, which is a rare hereditary disease. However, the detection of rare mutations using commonly used genetic tests is highly challenging. In the present study, next-generation sequencing (NGS) was used to identify a novel 7-bp deletion α-thalassemia in one individual from a Chinese family. Hematological parameters of the family members were determined using an automated cell counter, and hemoglobin electrophoresis was performed using a capillary electrophoresis system. Subsequently, NGS was performed on the genomic DNA of the patient and her family members. The 7-bp deletion (named Hb Honghe [HBA1: c.401_407delGCACCGT]) of α-thalassemia in the α-globin gene was confirmed using Sanger sequencing. The patient's father was also a heterozygous carrier of HBA1: c.401_407delGCACCGT deletion, but not her mother or sister. The application of the combined molecular approach is essential for the accurate diagnosis of rare thalassemia. This study reports a novel case of α- thalassemia. The characterization of the mutation might provide new insights into genetic counseling and accurate diagnosis of thalassemia.

Keywords: Chinese family; deletion mutation; next-generation sequencing; α-globin gene cluster; α-thalassemia.

MeSH terms

  • East Asian People
  • Gene Deletion
  • Glycated Hemoglobin
  • Humans
  • Multigene Family
  • Mutation
  • alpha-Globins / genetics
  • alpha-Thalassemia* / diagnosis
  • alpha-Thalassemia* / genetics

Substances

  • alpha-Globins
  • Glycated Hemoglobin