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目的 观察阵发性睡眠性血红蛋白尿症 (PNH)、再生障碍性贫血 (AA ,再障 )和骨髓增生异常综合征 (MDS)患者外周血粒细胞三种GPI 锚蛋白 (GPI AP)CD55、CD59和CD87的表达情况 ,测定血清可溶性尿激酶型纤溶酶原激活物受体 (su PAR)水平 ,探讨它们的临床意义。方法 PNH组 2 2例 ,其中4例合并血栓性疾病 ,5例为AA PNH综合征 ;再障组 30例 ,其中重型再障 9例 ,慢性再障 2 1例 ;MDS RA组 2 7例 ;健康对照组 2 0名。以流式细胞术检测外周血粒细胞CD55、CD59和CD87的表达 ,ELISA法检测血清su PAR。结果 2 0名健康人外周血粒细胞三种GPI AP阳性比例均大于 90 % ,CD59稳定性最好。PNH组三种GPI AP的阳性比例均显著下降 ,血清su PAR水平显著升高 ,与正常对照组比较 ,均有显著性差异。其中 ,频发组较不发作组三种GPI AP的表达率均明显降低 ,CD55的差异有显著性。PNH患者中 ,血栓阳性组较血栓阴性组CD87的表达率降低有显著性 ,血清su PAR水平显著升高。再障组粒细胞CD87表达率较正常对照组显著降低。 5例AA PNH综合征患者三种GPI AP的表达率与PNH患者比较 ,均无显著性差异 ;与再障患者比较 ,均显著降低。MDS RA组三种GPI AP的表达率较健康对照组均无显著性差异。结论 以流式细胞术检测外周血粒细胞CD55、CD59和CD
Objective To observe the expressions of three GPI-anchored proteins (GPI-APs) CD55 and CD59 in peripheral blood granulocyte in patients with paroxysmal nocturnal hemoglobinuria (PNH), aplastic anemia (AA) and myelodysplastic syndrome And CD87 expression levels, serum soluble urokinase-type plasminogen activator receptor (su PAR) levels, to explore their clinical significance. Methods A total of 22 patients with PNH were enrolled in this study. Among them, 4 patients had thrombotic disease and 5 patients had AA PNH syndrome. Aplastic anemia was also found in 30 patients, including 9 patients with severe aplastic anemia, 21 patients with chronic aplastic anemia and 27 patients with MDS RA. Healthy control group 20. The expression of CD55, CD59 and CD87 in peripheral blood granulocytes was detected by flow cytometry. The serum su PAR was detected by ELISA. Results The positive rates of GPI APs in 20 healthy human peripheral blood granulocytes were all above 90%, and the stability of CD59 was the best. The positive proportion of the three GPI APs in PNH group decreased significantly, while the level of su PAR in serum increased significantly. Compared with the normal control group, there was significant difference. Among them, the expression of three kinds of GPI AP in the frequent group was significantly lower than that in the non-episode group, and the difference of CD55 was significant. In patients with PNH, the expression of CD87 in the thrombus-positive group was significantly lower than that in the thrombus-negative group, and the level of serum su PAR was significantly increased. The expression of CD87 in aplastic anemia group was significantly lower than that in normal control group. The expression rates of three GPI APs in 5 AA patients with AAH syndrome were not significantly different from those in PNH patients, and were significantly lower than those in patients with aplastic anemia. The expression rates of three GPI APs in MDS RA group were not significantly different from those in healthy control group. Conclusions Peripheral blood neutrophils CD55, CD59 and CD were detected by flow cytometry