再生障碍性贫血和阵发性睡眠性血红蛋白尿血细胞GPI-AP缺陷分析

目的：探讨外周血细胞糖基磷脂酰肌醇锚蛋白（GPI－AP）缺陷与再生障碍性盆血（AA）和阵发性睡眠性血红蛋白尿（PNH）的关系。方法：用REDQUANT CD55／CD59,CELLQUANTCD55／CD59试剂盒和流式细胞术测15例正常人,47例AA和42例PNH或AA－PNH综合征患者外周血细胞GPI－AP的表达,结果：47例AA中16例（34．04％）血细胞CD55和CD59表达不同程度降低,且缺陷细胞百分率明显你芋AA－PNH综合征和PNH患者（P＜0．01）,结论：AA,AA－PNH及PNH患者外周血细胞存在不同程度的GPI－AP缺乏,缺陷细胞百分率检测可作为相关疾病诊断与转化的参考。

Glycosylphosphatidylinositol-anchored protein(GPI-AP)deficiency on blood cells of patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria

Objective To investigate the deficiency of glycosylphosphatidylinositol-anchored pro-teins(GPI-AP) in patients with aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria(PNH). Methods Flow cytometry (FCM), REDQUANT CD55/CD59 and CELLQUANT CD55/C59 kits were used to assess the expression of CD55 and CD59 on peripheral erythrocytes and granulocytes of 47 AA patients and 42 PNH or AA-PNH patients. Results A variant degree of deficiency of GPI-AP expression was found on granulocytes and/or erythrocytes in 16/47 (34.04 % ) AA patients, and the positivity of deficient cells was significantly lower than PNH and AA-PNH( P < 0.01) . Conclusion Varying degrees of GPI-AP frequently existed in AA, PNH and AA-PNH.The ratio of deficient cells was implicated in the differentiation of the related diseases and disease transformation.