Li Xuejin, Dao Ming, Lykotrafitis George, Karniadakis George Em
Division of Applied Mathematics, Brown University, Providence, RI 02912, USA.
Department of Materials Science and Engineering, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.
J Biomech. 2017 Jan 4;50:34-41. doi: 10.1016/j.jbiomech.2016.11.022. Epub 2016 Nov 12.
Sickle cell anemia (SCA) is an inherited blood disorder that causes painful crises due to vaso-occlusion of small blood vessels. The primary cause of the clinical phenotype of SCA is the intracellular polymerization of sickle hemoglobin resulting in sickling of red blood cells (RBCs) in deoxygenated conditions. In this review, we discuss the biomechanical and biorheological characteristics of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology and pathogenesis of SCA. Additionally, we highlight the adhesive heterogeneity of RBCs in SCA and their specific contribution to vaso-occlusive crisis.
镰状细胞贫血(SCA)是一种遗传性血液疾病,由于小血管的血管阻塞而导致疼痛性危象。SCA临床表型的主要原因是镰状血红蛋白在细胞内聚合,导致红细胞(RBC)在脱氧条件下发生镰变。在本综述中,我们讨论了镰状红细胞和镰状血液的生物力学和生物流变学特征,以及它们对更好地理解SCA的病理生理学和发病机制的意义。此外,我们强调了SCA中红细胞的黏附异质性及其对血管阻塞性危象的具体作用。