Wambua Sammy, Mwacharo Jedidah, Uyoga Sophie, Macharia Alexander, Williams Thomas N
Kenya Medical Research Institute/Wellcome Trust Programme, Centre for Geographic Medicine Research, Coast, PO Box 230, Kilifi District Hospital, Kilifi, Kenya.
Br J Haematol. 2006 Apr;133(2):206-9. doi: 10.1111/j.1365-2141.2006.06006.x.
Both the sickle cell trait (HbAS) and alpha(+)-thalassaemia are common in many tropical areas. While their individual haematological effects are well described, few studies describe their effects when inherited together. We present data from the Kenyan coast, which suggest that HbAS and alpha(+)-thalassaemia may interact to produce specific effects on haematological parameters. Overall, the difference in Hb concentrations between non-thalassaemics (alphaalpha/alphaalpha) and alpha(+)-thalassaemia homozygotes (-alpha/-alpha) was greater in non-HbAS (HbAA) (0.63 g/dl) than in HbAS children (0.25 g/dl). HbAS also ameliorated both the reduced mean cell volume and mean cell haemoglobin normally associated with the -alpha/-alpha genotype. Potential mechanisms and implications are discussed.
镰状细胞性状（HbAS）和α（+）-地中海贫血在许多热带地区都很常见。虽然它们各自的血液学影响已有详细描述，但很少有研究描述它们共同遗传时的影响。我们展示了来自肯尼亚海岸的数据，这些数据表明HbAS和α（+）-地中海贫血可能相互作用，对血液学参数产生特定影响。总体而言，非地中海贫血者（αα/αα）和α（+）-地中海贫血纯合子（-α/-α）之间的血红蛋白浓度差异，在非HbAS（HbAA）人群中（0.63 g/dl）比在HbAS儿童中（0.25 g/dl）更大。HbAS还改善了通常与-α/-α基因型相关的平均红细胞体积和平均红细胞血红蛋白的降低。文中讨论了潜在的机制和影响。
