Raghunathan Vikram M, Whitesell Peter L, Lim Seah H
Department of Medicine, Brown University Warren Alpert Medical School, Providence, RI, USA.
Department of Medicine, Howard University College of Medicine, Washington, DC, USA.
Ann Hematol. 2018 May;97(5):755-762. doi: 10.1007/s00277-017-3199-z. Epub 2017 Dec 6.
Sickle cell disease is one of the most common hereditary hemoglobinopathies worldwide, and its vaso-occlusive and hemolytic crises cause considerable patient morbidity. A growing body of evidence has shown that sleep-disordered breathing, and in particular, obstructive sleep apnea, occurs at high frequency in the sickle cell population, and that there is significant overlap in the underlying pathophysiology of these two conditions. Through a variety of mechanisms including nocturnal hypoxemia and increased oxidative stress, production of pro-inflammatory cytokines, and endothelial dysfunction, sickle cell anemia and sleep-disordered breathing potentiate each other's clinical effects and end-organ complications. Here, we will review the shared pathophysiologic mechanisms of these conditions and discuss their clinical sequelae. We will also examine the results of studies that have been carried out with clinical intervention of nocturnal hypoxemia in patients with sickle cell disease in the attempts to overcome the complications of the disease. Finally, we will propose the areas of investigation that merit further investigations in future in patients with sickle cell disease and sleep-disordered breathing.
镰状细胞病是全球最常见的遗传性血红蛋白病之一，其血管阻塞性和溶血性危象会导致患者出现相当严重的发病情况。越来越多的证据表明，睡眠呼吸障碍，尤其是阻塞性睡眠呼吸暂停，在镰状细胞病患者中高发，并且这两种病症在潜在病理生理学方面存在显著重叠。通过包括夜间低氧血症、氧化应激增加、促炎细胞因子产生以及内皮功能障碍等多种机制，镰状细胞贫血和睡眠呼吸障碍相互增强彼此的临床效应和终末器官并发症。在此，我们将回顾这些病症共同的病理生理机制，并讨论其临床后果。我们还将审视针对镰状细胞病患者夜间低氧血症进行临床干预以试图克服该疾病并发症的研究结果。最后，我们将提出未来在镰状细胞病和睡眠呼吸障碍患者中值得进一步研究的领域。
