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

作　　者：马宏杰[1] 贾国荣[1] 高丽君[2] 苏燕[2] 卢艳[1] 李喆[1] 李静[1] 贺其图[1] 秦文斌[2] 

机构地区：[1]包头医学院第一附属医院血液科 [2]包头医学院血红蛋白研究室,内蒙古包头014010

出　　处：《包头医学院学报》2015年第4期13-15,共3页Journal of Baotou Medical College

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。Objective：To explore the release of Hb from red blood cell （RBC） of patients with hereditary spherocytosis（HS）, with the appli-cation 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＆#39;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 HbA2 were by and large almost not separated from diagonal and MHA appeared in whole blood.There was no re-release from patients＆#39;RBC and whole blood in the whole blood multicomponent electrophore-sis.The isotonic and hypotonic whole journey release electrophoresis showed that the re-release was weakened obviously or disappeared from pa-tients＇RBC and whole blood.Conclusion：The obvious abnormality of RBC of patients with HS is demonstrated from the angle of electrophoretic re-lease and it is found that the fast moving Hb in patients＆#39;RBC is decreased obviously or deleted.

关 键 词：遗传性球形红细胞增多症 红细胞 快泳血红蛋白 HEREDITARY spherocytosis(HS) Red blood cell (RBC) HEMOGLOBIN release test(HRT) 

分 类 号：R555.1[医药卫生—血液循环系统疾病]