A comparative study of hematological parameters of alpha and beta thalassemias in a high prevalence zone: Saudi Arabia.
A comparative study of hematological parameters of alpha and beta thalassemias in a high prevalence zone: Saudi Arabia.
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DOI：
10.4103/0971-6866.92106
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发表时间：
2011-09-01
期刊：
影响因子：
--
通讯作者：
Al Dahmash, Badr Abdullah
中科院分区：
文献类型：
--
作者：
Mehdi, Syed Riaz;Al Dahmash, Badr Abdullah
背景和目的：沙特阿拉伯属于地中海贫血的高发区。地中海贫血类型的早期筛查对于进一步调查和管理至关重要。根据红细胞指标及其他血液学指标进行地中海贫血的分型研究。材料与方法：对沙特阿拉伯利雅得的991例地中海贫血临床疑似病例进行研究。对Coulter STKS血液学参数进行了研究。对所有血样进行醋酸纤维素血红蛋白电泳和高效液相色谱分析。基因缺失研究采用限制性内切酶Bam - HI进行限制性片段长度多态性（RFLP）技术。统计分析：采用SPSS 11.5版本进行统计分析。结果：血红蛋白电泳和基因分析结果显示，成人和儿童分别有406例（40.96%）和59例（5.95%）患有-地中海贫血特征性和重度。观察到各种缺失型α -地中海贫血426例。小细胞增多是β -地中海贫血特征和（- α /- α）和（- / α α）型α -地中海贫血的共同特征。MCH是地中海贫血中更显著的区别特征。重度β地中海贫血和α地中海贫血（- α / α α）的血液学参数几乎正常。结论：MCV和RBC计数在区分α和β地中海贫血方面没有统计学意义。有必要在区分地中海贫血与缺铁性贫血的区别指数基础上，开发一种区分α和β地中海贫血特征的区分指数。
BACKGROUND AND AIMS: Saudi Arabia falls in the high prevalent zone of alphaalpha and beta thalassemias. Early screening for the type of thalassemia is essential for further investigations and management. The study was carried out to differentiate the type of thalassemia based on red cell indices and other hematological parameters.MATERIALS AND METHODS: The study was carried out on 991 clinically suspected cases of thalassemias in Riyadh, Saudi Arabia. The hematological parameters were studied on Coulter STKS. Cellulose acetate hemoglobin electrophoresis and high-performance liquid chromatography (HPLC) were performed on all the blood samples. Gene deletion studies were carried out by restriction fragment length polymorphism (RFLP) technique using the restriction endonucleases Bam HI.STATISTICAL ANALYSIS: Statistical analysis was performed on SPSS 11.5 version.RESULTS: The hemoglobin electrophoresis and gene studies revealed that there were 406 (40.96%) and 59 (5.95 %) cases of beta thalassemia trait and beta thalassemia major respectively including adults and children. 426 cases of various deletion forms of alpha thalassemias were seen. Microcytosis was a common feature in beta thalassemias trait and (-alpha/-alpha) and (--/alphaalpha) types of alpha thalassemias. MCH was a more significant distinguishing feature among thalassemias. beta thalassemia major and alpha thalassemia (-alpha/alphaalpha) had almost normal hematological parameters.CONCLUSION: MCV and RBC counts are not statistically significant features for discriminating between alpha and beta thalassemias. There is need for development of a discrimination index to differentiate between alpha and beta thalassemias traits on the lines of discriminatory Indices available for distinguishing beta thalassemias trait from iron deficiency anemia.