阵发性睡眠性血红蛋白尿症、再生障碍性贫血和骨髓增生异常综合征患者三种GPI-锚蛋白的表达及临床意义  

Expression of three kinds of GPI-anchor proteins in paroxysmal nocturnal hemoglobinuria, aplastic anemia and myelodysplastic syndromes patients and their clinical implications

作　　者：董孝媛[1] 徐从高[1] 孙国瑞[2] 张锑[1] 彭军[1] 

机构地区：[1]山东大学齐鲁医院血液科,济南250012 [2]山东大学齐鲁医院普外科,济南250012

出　　处：《中华血液学杂志》2004年第4期198-201,共4页Chinese Journal of Hematology

基　　金：山东省自然科学基金资助项目 (Y2 0 0 2C0 7)

摘　　要：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。Objective To investigate the expressions of three kinds of glycosyl-phosphatidylinositol anchor proteins(GPI-AP),the CD 55, CD 59 and CD 87 on the peripheral granulocytes and the soluble u-PAR(su-PAR)level in serum from patients with paroxysmal nocturnal hemoglobinuria(PNH),aplastic anemia (AA),and myelodysplastic syndromes (MDS), and to analyse their clinical implications. Methods Twenty-two PNH patients, including 4 complicated with thrombotic diseases and 5 with AA-PNH syndrome, 30 AA patients, including 9 being severe AA (SAA) and 11 chronic AA (CAA), 27 MDS-RA patients, and 20 healthy individuals were tested. The expressions of CD 55, CD 59 and CD 87 on peripheral granulocytes were analyzed with flow cytometry. Serum su-PAR was assayed by ELISA. Results The CD 55+, CD 59+ and CD 87+ granulocytes in peripheral blood of 20 normal controls were all more than 90%. The expressions of three kinds of GPI-APs in 22 PNH patients were significantly decreased as compared with that in normal controls, AA patients and MDS-RA patients. The serum level of su-PAR in PNH group was higher than that of the other three groups. The expression of CD 87 was significantly decreased in thrombotic PNH patients as compared with that in non-thrombotic PNH patients. The expression of CD 87 was significantly decreased in AA patients than in normal controls. The expressions of three kinds of GPI-APs in 5 AA-PNH syndrome patients were remarkably reduced as compared with AA patients, but no significant difference was found for these indexes between AA-PNH syndrome and PNH patients and between 27 MDS-RA patients and 20 normal controls. Conclusion Measurement of CD 55, CD 59 and CD 87 expressions levels on the peripheral granulocytes and su-PAR in serum would be alternative approaches for diagnosis and differential diagnosis of PNH. Serum level of su-PAR may be helpful to monitor thrombosis in PNH patients.

关 键 词：阵发性睡眠性血红蛋白尿症 再生障碍性贫血 骨髓增生异常综合征 GPI-锚蛋白 临床意义 流式细胞术 

分 类 号：R695.2[医药卫生—泌尿科学] R556.5[医药卫生—外科学]