Coates Thomas D, Chalacheva Patjanaporn, Zeltzer Lonnie, Khoo Michael C K
Section of Hematology, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Department of Biomedical Engineering, Viterbi School of Engineering, University of Southern California, Los Angeles, CA, USA.
Clin Hemorheol Microcirc. 2018;68(2-3):251-262. doi: 10.3233/CH-189011.
Sickle cell disease (SCD) is a genetic disorder of hemoglobin producing hemoglobin-S (HbS) and resulting in recurrent severe episodes of pain, organ damage and premature death due to vaso- occlusion. Deoxy HbS polymerizes, causing red cells to become rigid and lodge in the microvasculature if they do not escape into larger vessels before this transformation occurs. The mechanism that triggers this transition from steady state to vaso-occlusive crisis (VOC) is not known. Patients state that cold, emotional stress, and pain itself can trigger these events. In spite of the connection between these symptoms and the autonomic nervous system (ANS), and the fact that the ANS regulates regional microvascular blood flow, the role of the ANS in sickle pathophysiology has not been significantly investigated. We will briefly review the mechanism of SCD vaso-occlusion, the dysautonomia associated with SCD and sickle trait, and the role that the ANS may play in the genesis of sickle vaso-occlusive crisis.
镰状细胞病（SCD）是一种血红蛋白的遗传性疾病，可产生血红蛋白-S（HbS），并由于血管阻塞导致反复出现严重的疼痛发作、器官损伤和过早死亡。脱氧HbS会聚合，导致红细胞变硬，如果在这种转变发生之前它们没有逃逸到较大的血管中，就会滞留在微血管中。引发从稳态转变为血管闭塞性危机（VOC）的机制尚不清楚。患者表示，寒冷、情绪压力和疼痛本身都可能引发这些事件。尽管这些症状与自主神经系统（ANS）之间存在联系，而且ANS可调节局部微血管血流，但ANS在镰状细胞病理生理学中的作用尚未得到充分研究。我们将简要回顾SCD血管阻塞的机制、与SCD和镰状细胞性状相关的自主神经功能障碍，以及ANS在镰状血管闭塞性危机发生过程中可能发挥的作用。
