出血性血小板病患者凝血酶受体GPIb／IX／V复合物表达研究

目的观察出血性血小板病患者的血小板膜糖蛋白（GP）Ib／IX／V复合物及其组分GP Ibα的表达。方法采用流式细胞术检测68例出血性血小板病患者在出血期的血小板GPIb／IX／V复合物和GP Ibα的表达，采用比浊法检测其血小板最大聚集率（MA），并与33例出血控制者及32例正常健康者比较。结果出血性血小板病患者在出血期间GPIb／IX／V复合物、GP Ibα表达明显低于正常组（P均〈0．01），出血控制者的GPIb／IX／V复合物、GP Ibα表达与正常组比较无显著性差异（P均〉0．05）。结论血小板GPIb／Ⅸ／V复合物作为凝血酶和血管性血友病因子的受体，在出血性血小板病患者体内的表达下降，导致血小板聚集功能障碍，可能是本病反复发作出血倾向的重要原因之一。

Expression of Platelet Membrane Glycoprotein Ib/IX/V , a Platelet Membrane Receptor, in Patients with Hemorrhagic Thrombopathy

Objective To investigate the expression differences of platelet membrane glycoprotein （GP） Ib/IX/V complex and its component GP Ibct between patients with hemorrhagic thrombopathy and healthy subjects. Methods The expressions of GP Ib/IX/V complex and GP Ibα were assessed by flow cytometry, and platelet maximum agglutination （MA） was determined by turbidity method in 68 bleeding patients with hemorrhagic thrombopathy and were compared with those in 33 bleeding controlled patients with hemorrhagic thrombopathy and 32 normal healthy subjects. Results The mean fluorescence intensity of GP Ib/IX/V complex and GP Ibct were lower in bleeding patients with hemorrhagic thrombopathy than those in the healthy subjects（P 〈0.01 ）, and there were no significant differences in the bleeding controlled patients with hemorrhagic thrombopathy and normal healthy subjects （ P 〉 0.05 ）. Conclusion As a receptor of thrombin and yon Willebrand factor, GP Ib/IX/V complex decreased in hemorrhagic thrombopathy patients, resulting in platelet aggregation dysfunction, which might partly explain the repeatedly paroxysmal hemorrhagic tendency in hemorrhagic thrombopathy.