Haemoglobin F, A2, and S levels in subjects with or without sickle cell trait in south-eastern Gabon

Background: Infant mortality due to sickle cell disease in sub-Saharan Africa is high, necessitating a better understanding of the modulating factors of the disease in this region. Methods: We assessed the hereditary persistence of foetal haemoglobin and α-thalassemia. We diagnosed 787 subjects, wit...

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Veröffentlicht in:Hematology (Luxembourg) 2017-09, Vol.22 (8), p.508-513
Hauptverfasser: Mombo, Landry-Erik, Mabioko-Mbembo, Gaël, Kassa-Kassa, Roland-Fabrice, Ontsitsagui, Emmanuel, Mboui-Ondo, Statiana, Nzé-Kamsi, Leatitia, Nkoghé, Dieudonné, Elion, Jacques
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Sprache:eng
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Zusammenfassung:Background: Infant mortality due to sickle cell disease in sub-Saharan Africa is high, necessitating a better understanding of the modulating factors of the disease in this region. Methods: We assessed the hereditary persistence of foetal haemoglobin and α-thalassemia. We diagnosed 787 subjects, with or without sickle cell trait, by capillary electrophoresis in the Medical Diagnostic Laboratory of the CIRMF (Franceville, Gabon). Results: Heterocellular and pancellular forms of hereditary persistence of foetal haemoglobin occurred at low rates of 10.9 and 2.3%, respectively. The distribution of HbS levels in individuals with sickle cell trait was trimodal, showing a high percentage (52.4%) of heterozygous subjects with α-thalassemia. The distribution of HbA2 levels was bimodal in individuals without sickle cell trait, estimated to be comprised of 12 and 15% of α and β-thalassemic heterozygous subjects, respectively. Conclusions: In sub-Saharan Africa, α-thalassemia is a far more prevalent modulating factor than hereditary persistence of foetal haemoglobin. Our study highlights the need for further investigation of thalassemia, haemoglobinopathies that are neglected in sub-Saharan Africa.
ISSN:1024-5332
1607-8454
DOI:10.1080/10245332.2017.1292622