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Hemoglobins F, A 2 , and E levels in Laotian children aged 6‐23 months with Hb E disorders: Effect of age, sex, and thalassemia types
Author(s) -
Kingchaiyaphum Benchawan,
Sanchaisuriya Kanokwan,
Fucharoen Goonnapa,
Chaibunruang Attawut,
Hess Sonja Y.,
Hinnouho GuyMarino,
Barffour Maxwell A.,
Wessells Kimbery R.,
Kounnavong Sengchanh,
Fucharoen Supan
Publication year - 2020
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/ijlh.13164
Subject(s) - heterozygote advantage , thalassemia , hemoglobin , medicine , compound heterozygosity , gastroenterology , biology , genetics , genotype , mutation , gene
Determination of hemoglobins (Hbs) F, A 2, and E is crucial for diagnosis of thalassemia. This study determined the levels of Hbs F, A 2, 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 A 2 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 A 2 = 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 A 2 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 A 2 but not Hb E. Conclusions Our findings can guide the development of a diagnostic approach to thalassemia in children aged 6‐23 months.