Sickle cell vasculopathy: vascular phenotype on fire!
Sickle cell vasculopathy: vascular phenotype on fire!
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镰状细胞血管病：血管表型着火！
DOI：
10.1113/jp276705
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Kato,GregoryJ
中科院分区：
文献类型：
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作者：
Kato,GregoryJ
临床医生、科学家和普通人都知道镰刀形细胞在血液循环中流动不良。红细胞僵硬是镰状细胞血管闭塞的基础，但越来越多的证据表明，血管壁在一个亚组的患者中呈现典型的血管病变特征。镰状细胞贫血性卒中或肺动脉高压患者的大动脉表现为内膜和中膜增生、不规则内皮伴原位血栓形成和管腔闭塞，其功能特征为血管功能障碍。肺动脉高压的确凿证据出现在大约10%的镰状细胞贫血患者中，在另外25%左右的患者中发现了较轻的发现。肺动脉高压发生在约6%，这是约2000倍，患病率在成人镰状细胞贫血比一般人群。镰状细胞性贫血是这些血管病变并发症发展的危险因素。这种闷烧风险被其他风险因素煽动起来，包括溶血强度、衰老、慢性肝脏或肾脏疾病和铁过载（Kato et al. 2017）。肺血管病变的多因素风险使人联想到一般人群中动脉粥样硬化血管危险因素的叠加效应。事实上，动脉粥样硬化的许多典型危险因素也与镰状细胞贫血中的肺血管病变相关，例如全身性高血压、低载脂蛋白AI表达、高脂血症和一氧化氮生物利用度受损。感谢本期《纽约时报》的一篇文章，
Clinicians, scientists and lay people alike grasp the concept that sickle-shaped cells flow poorly in the circulation. Red cell stiffness is the basis of sickle cell vaso-occlusion, but growing evidence shows that the blood vessel wall assumes classical features of vasculopathy in a subgroup of patients. Large arteries in sickle cell patients with ischaemic stroke or pulmonary arterial hypertension show intimal and medial hyperplasia, irregular endothelium with in situ thrombosis and luminal obliteration, functionally characterized by vasomotor dysfunction. Solid evidence for pulmonary hypertension turns up in approximately 10% of sickle cell anaemia patients, with milder findings in another 25% or so. Pulmonary arterial hypertension occurs in about 6%, which is about 2000-fold greater prevalence in adults with sickle cell anaemia than in the general population. Sickle cell anaemia is a risk factor for the development of these vasculopathic complications. This smouldering risk is fanned into flames by additional risk factors, including haemolytic intensity, ageing, chronic liver or kidney disease, and iron overload (Kato et al. 2017). This multifactorial risk for pulmonary vasculopathy is reminiscent of the additive effect of vascular risk factors for atherosclerosis in the general population. In fact, many typical risk factors for atherosclerosis are also associated with pulmonary vasculopathy in sickle cell anaemia, such as systemic hypertension, low apolipoprotein AI expression, hypertriglyceridaemia and impaired bioavailability of nitric oxide. Thanks to an article in this issue of The