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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 investigate hemoglobin release from erythrocytes in patients with hereditary spherocytosis using the hemoglobin release test created in our laboratory. Methods: A series of hemoglobin release tests were performed: one-way, two-way, two-way, and so on (at room temperature, 37 ℃). Results: The hemoglobin released by one-way electrophoresis in patients with erythrocytes was partially or significantly reduced. Two-dimensional electrophoresis results, patients with red blood cells in addition to fasting hemoglobin decreased or missing, but also visible red blood cells and whole blood Hb A2 diagonal distribution, and whole blood hematin albumin. Whole blood multi-component experiments can be seen in patients with red blood cells and no further release of whole blood. Hypertonic full-length electrophoresis showed that patients with red blood cells and whole blood re-release were significantly weakened or disappeared. Conclusion: From the point of view of electrophoresis release, the erythrocytes in patients were obviously abnormal. It was found that the hemoglobin in fast red blood cells in patients was significantly reduced or absent.