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

• Published in: Hematology (Luxembourg) Vol. 22; no. 8; pp. 508 - 513
• Main Authors: Mombo, Landry-Erik, Mabioko-Mbembo, Gaël, Kassa-Kassa, Roland-Fabrice, Ontsitsagui, Emmanuel, Mboui-Ondo, Statiana, Nzé-Kamsi, Leatitia, Nkoghé, Dieudonné, Elion, Jacques
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis 14.09.2017
• Subjects: Adolescent; Child; Electrophoresis, Capillary; Erythrocyte Indices; Female; Fetal Hemoglobin - metabolism; Gabon; HbA2; HbF; HbS; Hemoglobin A2 - metabolism; Hemoglobin, Sickle - metabolism; Humans; Infant; Male; Pregnancy; Rural Population; sickle cell trait; Sickle Cell Trait - blood; Sickle Cell Trait - diagnosis; Sickle Cell Trait - epidemiology; thalassemia; Young Adult; HbS; Gabon; HbA2; thalassemia; sickle cell trait; HbF
• ISSN: 1024-5332; 1607-8454
• DOI: 10.1080/10245332.2017.1292622

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.