【摘要】 目的 探讨外周血细胞糖基磷脂酰肌醇锚蛋白(ＧＰＩ－ＡＰ)缺陷与再生障碍性贫血(ＡＡ)和阵发性睡眠性血红蛋白尿(ＰＮＨ)的关系。方法 用ＲＥＤＱＵＡＮＡＮＴ ＣＤ５５/ＣＤ ５９、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低于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。更多还原

【Abstract】 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.更多还原