Wautier J L
Ann Med Interne (Paris). 1986;137(6):477-9.
The association of erythrocyte disorders and thrombosis in polycythemia vera, paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell anemia suggest that erythrocyte abnormality, could be involved in the genesis of thrombosis. A significant correlation between the hematocrit level and the incidence of thrombosis has been reported indicating the role of blood viscosity, but did to exclude an intrinsic red cell. Thrombotic episodes in sickle cell anemia might be explained by a decreased deformability and an increased adhesion of red cells to endothelium. The incidence of thrombosis is of 40 p. 100 in patient with PNH with a frequent sus-hepatic localization. In addition to abnormal erythrocyte adhesion, abnormal membrane lipid distribution was observed in PNH, suggesting that they could participate in the pathophysiology of thrombosis.
真性红细胞增多症、阵发性夜间血红蛋白尿（PNH）和镰状细胞贫血中红细胞疾病与血栓形成的关联表明，红细胞异常可能参与了血栓形成的发生过程。据报道，血细胞比容水平与血栓形成发生率之间存在显著相关性，这表明了血液粘度的作用，但并未排除红细胞内在因素。镰状细胞贫血中的血栓形成发作可能是由于红细胞变形性降低以及红细胞与内皮细胞的粘附增加所致。PNH患者的血栓形成发生率为40%，且常发生于肝窦部位。除了异常的红细胞粘附外，在PNH中还观察到膜脂质分布异常，这表明它们可能参与了血栓形成的病理生理过程。
