Horne M K
Am J Med. 1981 Feb;70(2):288-98. doi: 10.1016/0002-9343(81)90764-6.
Sickle cell anemia represents an aberration of blood rheology due to a loss of normal red cell deformability. The characteristically low hematocrit compensates for the stiffness of the sickle cells, leaving the patient with approximately normal whole blood viscosity. However, the microvascular flow of sickle cell blood is constantly jeopardized by hemoglobin gellation due to hypoxemia. The cells containing the highest concentration of hemoglobin S are the most viscous and are at the greatest risk for abrupt sickling. Successful treatment of this disease will require interruption of the basic pathogenetic mechanisms and preservation of normal blood rheology.
镰状细胞贫血是由于正常红细胞变形性丧失而导致的血液流变学异常。特征性的低血细胞比容补偿了镰状细胞的僵硬性，使患者的全血粘度大致正常。然而，由于低氧血症，镰状细胞血液的微血管血流不断受到血红蛋白凝胶化的威胁。含有最高浓度血红蛋白S的细胞粘性最大，突然镰变的风险也最大。成功治疗这种疾病需要中断基本的发病机制并保持正常的血液流变学。
