Coinheritance of Β-Thalassemia and Sickle Cell Anaemia in Southwestern Nigeria

Abstract

BACKGROUND: Genes for haemoglobin S are found in high frequencies in Nigeria. However, there islittle information on beta thalassemia in sickle cell anaemia in this population. The clinical presentationof HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a severe disordersimilar to homozygous sickle cell disease.MATERIALS AND METHODS: Haemoglobin A 2 and HbF were determined in sickle cell anaemiapatients attending LAUTECH Teaching Hospital, Osogbo, by elution after electrophoresis and alkalinedenaturation methods respectively. Haematological parameters were estimated using Sysmex KX-21Nand percentage target cells using Leishman’s staining technique.RESULTS: Exactly 6% f the SCA patients were found to have elevated HbA 2 (>3.3%) and HbF (>1.3%).These patients also had normal erythrocyte indices, increased platelet count, a significantly higher HCTand an increased % target cell.CONCLUSION: These findings confirm that the frequency of beta thalassaemia in sickle cell patients inNigeria is higher than previously thought. It is therefore important to consider the possibility of thisvariant in patients with sickle cell anaemia since their course may differ from that of patients withhomozygous sickle cell anaemia.