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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。
OBJECTIVE: To detect the expression of urokinase-type plasminogen activator receptor in patients with paroxysmal nocturnal hemoglobinuria (PNH) and to explore its relationship with PNH diagnosis and thrombosis. Methods: Twenty-two patients with PNH, including 4 with thrombosis; 20 with simple heart / thrombosis; and 20 with healthy volunteers. The expression of CD87 on peripheral blood neutrophils was detected by flow cytometry. Serum soluble urokinase receptor (su-PAR) was detected by ELISA. Results: The percentage of CD87 + granulocytes in peripheral blood of healthy volunteers were all> 90%. There was no significant difference in expression rate between simple heart / cerebral thrombosis group and healthy volunteers. Compared with the other two groups, the percentage of CD87 + granulocytes in peripheral blood of PNH patients was significantly lower (P <0.01). The levels of serum su PAR in PNH group were significantly higher than those in non-thrombosis group (P <0.01). The proportion of CD87 + granulocytes in peripheral blood of patients with PNH was significantly lower (P <0.01). There was a negative correlation between serum su PAR and percentage of CD87 + granulocytes in patients with PNH. Conclusion: The percentage of CD87 + granulocytes in peripheral blood of patients with PNH is significantly lower, the level of serum su PAR is significantly higher, which is correlated with the complication of thrombosis. Detection of granulocyte CD87 and serum su PAR by flow cytometry is helpful for the diagnosis of PNH and monitoring the change of thrombosis.