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

Benchawan Kingchaiyaphum, Kanokwan Sanchaisuriya, Goonnapa Fucharoen, Attawut Chaibunruang, Sonja Y Hess, Guy-Marino Hinnouho, Maxwell A Barffour, Kimbery R Wessells, Sengchanh Kounnavong, Supan Fucharoen, Benchawan Kingchaiyaphum, Kanokwan Sanchaisuriya, Goonnapa Fucharoen, Attawut Chaibunruang, Sonja Y Hess, Guy-Marino Hinnouho, Maxwell A Barffour, Kimbery R Wessells, Sengchanh Kounnavong, Supan Fucharoen

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.

Conflict of interest statement

The authors have no competing interests.

© 2020 The Authors. International Journal of Laboratory Hematology published by John Wiley & Sons Ltd.

Figures

Figure 1
Figure 1
The distributions of Hb F, A2, and E levels in Laotian children aged 6‐23 mo; (A): distribution of Hb F among total participants, (B): distribution of Hb A2 among total participants, (C): distribution of Hb E among Hb E heterozygotes, and (D): distribution of Hb E among Hb E homozygotes; X‐axis represents levels of Hb F, A2, and E as indicated (expressed as % of total Hb); Y‐axis represents the number of cases [Colour figure can be viewed at wileyonlinelibrary.com]

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