Connes Philippe, Renoux Céline, Romana Marc, Abkarian Manouk, Joly Philippe, Martin Cyril, Hardy-Dessources Marie-Dominique, Ballas Samir K
Laboratoire LIBM EA7424, Team"Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France.
Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France.
Clin Hemorheol Microcirc. 2018;68(2-3):165-172. doi: 10.3233/CH-189005.
This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events. Although less studied, RBC aggregation properties are very different between SCA and healthy individuals and it was demonstrated that increased RBC aggregates strength could be involved in some complications. Finally, several studies have established that the vascular system of SCA patients could not fully compensate any increase in blood viscosity because of the loss of vascular reactivity, which may result in vaso-occlusive crises.
本综述聚焦于异常血液流变学在镰状细胞贫血（SCA）病理生理学中的作用。SCA的特征是红细胞（RBC）变形性降低，但这种降低在患者中差异很大。最近的研究表明，红细胞变形性最低（通过激光衍射法测量）的患者溶血增强，更容易出现诸如阴茎异常勃起、腿部溃疡和肾小球病等多种并发症。相比之下，红细胞变形性最高且未接受羟基脲治疗的患者似乎更频繁地发生血管闭塞样事件。虽然研究较少，但SCA患者与健康个体的红细胞聚集特性差异很大，并且已证明红细胞聚集体强度增加可能与某些并发症有关。最后，多项研究证实，由于血管反应性丧失，SCA患者的血管系统无法完全代偿血液粘度的任何增加，这可能导致血管闭塞性危机。
