阵发性睡眠性血红蛋白尿患者尿激酶受体的表达及临床意义

目的:检测尿激酶型纤溶酶原激活物受体在阵发性睡眠性血红蛋白尿(PNH)患者中的表达,并探讨其与PNH诊断以及血栓形成的关系。方法:PNH患者22例,其中4例伴血栓形成;单纯心/脑血栓形成患者20例;健康志愿者20例。以流式细胞术检测外周血粒细胞上CD87的表达,ELISA法检测血清可溶性尿激酶受体(su鄄PAR)。结果:健康志愿者外周血CD87 粒细胞比例皆>90%,单纯心/脑血栓形成组与健康志愿者组表达率差异无统计学意义。与其他两组相比,PNH患者外周血CD87 粒细胞百分率显著降低(P<0.01)。PNH组血清su鄄PAR水平显著升高(P<0.01),PNH患者伴血栓形成组与不伴血栓形成组比较,前者外周血CD87 粒细胞比例明显降低,血清su鄄PAR水平显著升高(P<0.01)。PNH患者血清su鄄PAR与CD87 粒细胞百分率呈负相关。结论:PNH患者外周血CD87 粒细胞百分率显著降低,血清su鄄PAR水平明显增高,与血栓形成并发症相关。以流式细胞术检测粒细胞CD87及血清su鄄PAR,有助于PNH的诊断及监测血栓形成的病情变化。

Expression of urokinase-type plasminogen activator receptor in paroxysmal nocturnal hemoglobinuria and its clinical implications

Objective: To investigate the expression of urokinase-type plasminogen activator receptor (u-PAR, CD87) on the peripheral blood granulocytes and the soluble u-PAR (su-PAR) level in serum from patients with paroxysmal nocturnal hemoglobinuria (PNH) and to analyze its clinical implications. Methods: Twenty-two patients with PNH, 20 patients with simple thrombosis and 20 healthy volunteers were included in this study. The expression of CD87 on peripheral blood granulocytes were analyzed with flow cytometry. Serum su-PAR was assayed by enzyme-linked immunoadsorbent assay (ELISA). Results: All the fraction of CD87 granulocytes in peripheral blood of 20 healthy volunteers was more than 90%. The expression of CD87 in 22 patients with PNH decreased significantly and the level of serum su-PAR increased. In the 22 patients with PNH, the fraction of CD87 granulocytes decreased significantly and the serum level of su-PAR elevated significantly in patients with thrombosis than in patients without thrombosis. The serum level of su-PAR was negatively correlated to the percentage of CD87 granulocytes in patients with PNH. Conclusion: In patients with PNH, the expression of CD87 in granulocytes decreases remarkably and the serum level of su-PAR increases significantly. Flow cytometric analysis of CD87 on the peripheral blood granulocytes and detection of su-PAR are of value in diagnosis of PNH and helpful for monitoring thrombosis.