Hebbel R P, Eaton J W, Steinberg M H, White J G
Blood Cells. 1982;8(1):163-73.
Unconvinced that the pathophysiology of sickle-cell disease is fully explained by traditional rheologic considerations, we have searched for additional factors which might be implicated in the development of acute vasocclusive crises in this disease. Sickle RBC adhere abnormally to cultured human vascular endothelial cells, an abnormality requiring neither deoxygenation nor frank morphologic distortion of the RBC. This adherence appears to be caused by an aberrancy of RBC surface charge topography on sickle RBC. Propensity for RBC adherence individual. Among patients with sickle cell anemia, frequency of acute vasocclusive crises correlates significantly with RBC adherence to endothelium. Patients with the clinically less severe doubly heterozygous sickling disorders have a lesser RBC adherence to endothelium than do patients with sickle-cell anemia. RBC/endothelial interactions are modified by factors in the RBC's environment such as fibrinogen, providing a possible mechanism by which concurrent illness might predispose towards the development of vasocclusion. We hypothesize that sickle RBC adherence to endothelium is the factor which initiates acute vasocclusion in sickle-cell disease, either by primarily occluding small vessels or by slowing microvascular blood flow so that secondary, reversible RBC sickling can occur.
由于认为镰状细胞病的病理生理学不能完全用传统的血液流变学因素来解释，我们一直在寻找可能与该病急性血管闭塞性危象发生有关的其他因素。镰状红细胞异常粘附于培养的人血管内皮细胞，这种异常既不需要红细胞脱氧，也不需要其明显的形态畸变。这种粘附似乎是由镰状红细胞表面电荷拓扑结构异常引起的。红细胞的粘附倾向因人而异。在镰状细胞贫血患者中，急性血管闭塞性危象的发生频率与红细胞对内皮细胞的粘附显著相关。临床症状较轻的双重杂合子镰状细胞病患者的红细胞对内皮细胞的粘附程度低于镰状细胞贫血患者。红细胞与内皮细胞的相互作用会受到红细胞环境中的因素（如纤维蛋白原）的影响，这为并发疾病可能导致血管闭塞的发生提供了一种可能的机制。我们推测，镰状红细胞对内皮细胞的粘附是引发镰状细胞病急性血管闭塞的因素，其方式要么是主要阻塞小血管，要么是减缓微血管血流，从而导致继发性、可逆性红细胞镰变。
