Hemoglobins F, A2, and E levels in Laotian children aged 6‐23 months with Hb E disorders: Effect of age, sex, and thalassemia types
Open Access
- 12 February 2020
- journal article
- research article
- Published by Wiley in International Journal of Laboratory Hematology
- Vol. 42 (3) , 277-283
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
Funding Information
- Bill and Melinda Gates Foundation
This publication has 26 references indexed in Scilit:
- Molecular and hematological studies in a large cohort of α0-thalassemia in northeast Thailand: Data from a single referral centerBlood Cells, Molecules, and Diseases, 2013
- Phenotypic expression of hemoglobins A2, E and F in various hemoglobin E related disordersBlood Cells, Molecules, and Diseases, 2012
- Is HbA2level a reliable diagnostic measurement for β‐thalassemia trait in people with iron deficiency?American Journal of Hematology, 2011
- Genotype and phenotype characterizations in a large cohort of β-thalassemia heterozygote with different forms of α-thalassemia in northeast ThailandBlood Cells, Molecules, and Diseases, 2011
- Prevention of severe thalassemia in northeast Thailand: 16 years of experience at a single university centerPrenatal Diagnosis, 2010
- The Range of Hemoglobin A2 in Hemoglobin E Heterozygotes as Determined by Capillary ElectrophoresisAmerican Journal of Clinical Pathology, 2009
- Expression of E and -globin genes in infants heterozygous for hemoglobin E and double heterozygous for hemoglobin E and -thalassemiaHaematologica, 2007
- The Diverse Molecular Basis and Hematological Features of Hb H and AEBart’s Diseases in Northeast ThailandActa Haematologica, 2004
- Hb Paksé [(α2) CODON 142 (TAA→TATOR Term→Tyr)] IN THAI PATIENTS WITH EABart's DISEASE AND Hb H DISEASEHemoglobin, 2002
- Abnormal RNA processing due to the exon mutation of βE-globin geneNature, 1982