HbF in HbE/β-thalassemia: A clinical and laboratory correlation

Hematology. 2015 Jul;20(6):349-53. doi: 10.1179/1607845414Y.0000000203. Epub 2014 Sep 22.

Abstract

Introduction: Fetal hemoglobin (HbF) is the predominant hemoglobin in red cells during fetal life. Just after birth, the level of HbF decreases gradually to <1%, and is replaced mainly by adult hemoglobin (HbA) (∼ 97%). However, higher HbF levels could be associated with HbE/β-thalassemia, a complex thalassemia intermedia with a diverse clinical severity ranging from mild-to-severe anemia. This study investigates the correlation of HbF level with the clinical and laboratory data of HbE/β-thalassemia individuals.

Methods: Peripheral blood samples from 30 HbE/β-thalassemia subjects were subjected to a full blood count, genomic as well as quantitative real-time polymerase chain reaction gene expression studies. Statistical analyses were performed using SPSS 17.0.

Results: HbF levels were influenced by age, mean cell volume (MCV), mean cell hemoglobin (MCH), HbA, β-globin, and α/β-globin expressions.

Discussion: HbF production is affected by the α/β-globin chain imbalance due to the lack of β-globin gene expression as well as inversely correlates to the amount of functional hemoglobin available in the cells.

Keywords: HbE/β-thalassemia; HbF; MCH; MCV; β-globin expression.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Female
  • Fetal Hemoglobin / genetics*
  • Genotype
  • Hemoglobin E / genetics*
  • Humans
  • Male
  • Middle Aged
  • Young Adult
  • beta-Thalassemia / blood*

Substances

  • Hemoglobin E
  • Fetal Hemoglobin