Red cell genetic abnormalities in Peninsular Arabs: Sickle haemoglobin, G6PD deficiency, and α and β thalassaemia

Abstract

The frequencies of four major red cell genetic defects, sickle haemoglobin (Hb S), glucose 6 phosphate dehydrogenase deficiency (G6PD), and α and β thalassaemia, have been determined in nearly 5000 subjects from the three major Peninsula Arab States, namely Yemen (North and South), the United Arab Emirates, and Oman. All four defects are common with an overall pattern of α thalassaemia > G6PD deficiency > β thalassaemia > Hb A/S. However, the frequencies of these within each state varies and they are, respectively, Oman: 0.389, 0.328, 0.024, and 0.038; the United Arab Emirates: 0.165, 0.087, 0.017, and 0.019; and Yemen: 0.065, 0.062, 0.624, and 0.0095. Two, namely α thalassaemia and G6PD deficiency, are extremely common, but in spite of this there appears to be a lack of observed clinical disease. For example, Hb H disease and Barts hydrops fetalis were not seen and the oxidative haemolytic syndromes are rare.