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遗传性球形红细胞增多症患者红细胞内快泳血红蛋白缺失

Absence of fast moving Hb in RBC of patients with hereditary spherocytosis

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【作者】 马宏杰贾国荣高丽君苏燕卢艳李喆李静贺其图秦文斌

【Author】 MA Hongjie;JIA Guorong;GAO Lijun;SU Yan;LU Yan;LI Zhe;LI Jing;HE Qitu;QIN Wenbin;Department of Hematology,the First Affiliated Hospital of Baotou Medical College;Laboratory of Hemoglobin,Baotou Medical College;

【机构】 包头医学院第一附属医院血液科包头医学院血红蛋白研究室

【摘要】 目的:利用我室创建的血红蛋白释放试验,研究遗传性球形红细胞增多症患者红细胞释放血红蛋白情况。方法:采用一系列血红蛋白释放试验:单向释放电泳、双向释放电泳、全血多组分电泳和等低渗全程释放电泳(室温、37℃),比较研究患者与正常人红细胞的差异。结果:患者红细胞经单向电泳释放出的血红蛋白快泳部分缺失或明显减少。双向电泳结果,患者红细胞除快泳血红蛋白减少或缺失外,还可见红细胞及全血Hb A2沿对角线分布,而且全血出现高铁血红素白蛋白。全血多组分实验中可见,患者红细胞及全血均无再释放。等低渗全程电泳显示,患者红细胞及全血的再释放都明显减弱或消失。结论:从电泳释放角度证明患者的红细胞明显异常,发现患者红细胞内快泳血红蛋白明显减少或缺失。

【Abstract】 Objective: To explore the release of Hb from red blood cell( RBC) of patients with hereditary spherocytosis( HS),with the application of hemoglobin release test( HRT) established in our laboratory. Methods: To compare the difference in RBC between patients with HS and the normal individuals through a series of HRTs,including the unidimensional release electrophoresis,the two dimensional release electrophoresis,the whole blood multicomponent electrophoresis,and the isotonic and hypotonic whole journey release electrophoresis( room temperature,37 ℃).Results: The fast moving Hb released from patients’ RBC through the unidimensional electrophoresis was deleted or decreased obviously. The results of the two dimensional electrophoresis showed that besides that,RBC and whole blood Hb A2 were by and large almost not separated from diagonal and MHA appeared in whole blood. There was no re-release from patients’ RBC and whole blood in the whole blood multicomponent electrophoresis. The isotonic and hypotonic whole journey release electrophoresis showed that the re-release was weakened obviously or disappeared from patients’ RBC and whole blood. Conclusion: The obvious abnormality of RBC of patients with HS is demonstrated from the angle of electrophoretic release and it is found that the fast moving Hb in patients’ RBC is decreased obviously or deleted.

  • 【文献出处】 包头医学院学报 ,Journal of Baotou Medical College , 编辑部邮箱 ,2015年04期
  • 【分类号】R555.1
  • 【被引频次】3
  • 【下载频次】54
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