Hebbel R P, Moldow C F, Steinberg M H
Blood. 1981 Nov;58(5):947-52.
The abnormal adherence of sickle erythrocytes to cultured human vascular endothelium varies among patients and correlates with vasocclusive severity within and among the different sickling disorders. For individual patients, the development of an acute vasocclusive event is not accompanied by an increase in the inherent propensity for their erythrocytes to adhere to endothelium. However, the onset of vasocclusion appears to be associated with the appearance of plasma factors, such as fibrinogen, which enhance the adherence of sickle erythrocytes to endothelium. In addition, sickle erythrocytes adhere even more avidly to injured endothelium and under hyperosmolar conditions. Thus, the overall clinical severity of the sickling disorders appears to correlate with the inherent propensity for sickle erythrocytes to adhere to endothelium, and changes in the red cells' environment may help precipitate crises by the sudden facilitation of erythrocyte/endothelial interactions.
镰状红细胞与培养的人血管内皮细胞的异常黏附在患者之间存在差异,并且与不同镰状细胞疾病内部及之间的血管闭塞严重程度相关。对于个体患者而言,急性血管闭塞事件的发生并未伴随着其红细胞黏附内皮细胞的固有倾向增加。然而,血管闭塞的发作似乎与血浆因子(如纤维蛋白原)的出现有关,这些血浆因子会增强镰状红细胞与内皮细胞的黏附。此外,镰状红细胞在高渗条件下以及与受损内皮细胞的黏附更为强烈。因此,镰状细胞疾病的总体临床严重程度似乎与镰状红细胞黏附内皮细胞的固有倾向相关,红细胞环境的变化可能通过突然促进红细胞/内皮细胞相互作用而促使危机发生。