Coinheritance of B-Thalassemia and Sickle Cell Anaemia in Southwestern Nigeria.
Coinheritance of B-Thalassemia and Sickle Cell Anaemia in Southwestern Nigeria.
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DOI：
10.4314/ejhs.v26i6.3
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发表时间：
2016-11
影响因子：
1.2
通讯作者：
Saidat L
中科院分区：
文献类型：
--
作者：
Vincent O;Oluwaseyi B;James B;Saidat L
血红蛋白S基因在尼日利亚发现的频率很高。然而，在这一人群中，镰状细胞贫血中β地中海贫血的信息很少。HbS- β地中海贫血的临床表现差异很大，从无症状状态到类似于纯合子镰状细胞病的严重疾病。血红蛋白A2和HbF测定镰状细胞贫血患者参加LAUTECH教学医院，Osogbo，电泳后洗脱和碱性变性方法分别。使用Sysmex KX-21 N估计血液学参数，使用Leishman染色技术估计靶细胞百分比。SCA患者中有6%的患者HbA 2（>3.3%）和HbF（>1.3%）升高。这些患者还具有正常的红细胞指数、增加的血小板计数、显著更高的HCT和增加的%靶细胞。这些发现证实，在尼日利亚镰状细胞患者中β地中海贫血的发生率比以前认为的要高。因此，重要的是要考虑镰状细胞贫血患者中这种变异的可能性，因为他们的病程可能与纯合子镰状细胞贫血患者不同。
Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a severe disorder similar to homozygous sickle cell disease. Haemoglobin A2 and HbF were determined in sickle cell anaemia patients attending LAUTECH Teaching Hospital, Osogbo, by elution after electrophoresis and alkaline denaturation methods respectively. Haematological parameters were estimated using Sysmex KX-21N and percentage target cells using Leishman's staining technique. Exactly 6% f the SCA patients were found to have elevated HbA2 (>3.3%) and HbF (>1.3%). These patients also had normal erythrocyte indices, increased platelet count, a significantly higher HCT and an increased % target cell. These findings confirm that the frequency of beta thalassaemia in sickle cell patients in Nigeria is higher than previously thought. It is therefore important to consider the possibility of this variant in patients with sickle cell anaemia since their course may differ from that of patients with homozygous sickle cell anaemia.