Hemoglobins F, A2 , and E levels in Laotian children aged 6-23 months with Hb E disorders: Effect of age, sex, and thalassemia types

Int J Lab Hematol. 2020 Jun;42(3):277-283. doi: 10.1111/ijlh.13164. Epub 2020 Feb 12.

Abstract

Introduction: Determination of hemoglobins (Hbs) F, A2, and E is crucial for diagnosis of thalassemia. This study determined the levels of Hbs F, A2, and E in children aged 6-23 months and investigated the effect of age, sex, and types of thalassemia on the expression of these Hbs.

Methods: A total of 698 blood samples of Laotian children including 272 non-Hb E, 271 Hb E heterozygotes, and 155 Hb E homozygotes were collected. Hb profiles were determined using the capillary zone electrophoresis. Coinheritance of α-thalassemia and the homozygosity for Hb E mutation were checked by PCR-based assay.

Results: Children heterozygous and homozygous for Hb E had significantly higher Hb F and A2 levels than non-Hb E children (median Hb F = 1.1% for non-Hb E group, 2.7% for Hb E heterozygotes, and 9.4% for Hb E homozygotes; median Hb A2 = 2.6% for non-Hb E group, 3.8% for Hb E heterozygotes, and 5.2% for Hb E homozygotes). The median Hb E levels were 21.9% for Hb E heterozygotes and 85.3% for Hb E homozygotes. Comparing within group, there was a statistically significant difference between children with and without an α-gene defect for Hb A2 and E, but not Hb F. Based on a multiple regression analysis, age and sex were significantly associated with the expression of Hb F and A2 but not Hb E.

Conclusions: Our findings can guide the development of a diagnostic approach to thalassemia in children aged 6-23 months.

Keywords: hemoglobin A2; hemoglobin E; hemoglobin F; hemoglobinopathy; thalassemia; young children.

Publication types

  • Multicenter Study
  • Randomized Controlled Trial

MeSH terms

  • Age Factors
  • Female
  • Fetal Hemoglobin* / genetics
  • Fetal Hemoglobin* / metabolism
  • Hemoglobin A2* / genetics
  • Hemoglobin A2* / metabolism
  • Hemoglobin E* / genetics
  • Hemoglobin E* / metabolism
  • Heterozygote*
  • Homozygote*
  • Humans
  • Infant
  • Laos
  • Male
  • Sex Factors
  • alpha-Thalassemia* / blood
  • alpha-Thalassemia* / genetics

Substances

  • Hemoglobin A2
  • Hemoglobin E
  • Fetal Hemoglobin