Division of Endocrinology, Department of Internal Medicine, Polytechnic University of Marche Region, Ancona, Italy.
Neuroendocrinology. 2010;92 Suppl 1:55-9. doi: 10.1159/000314349. Epub 2010 Sep 10.
A hypercoagulable state and its consequent increased incidence of thromboembolic complications are reported in patients with Cushing's syndrome (CS). These alterations are related to cortisol excess that induces prothrombotic changes in blood by several and complex mechanisms including increased levels of clotting factors, mainly factor VIII and von Willebrand factor (VWF) and impaired fibrinolytic capacity. However, it has recently been observed that the increase in VWF levels is not a constant feature of CS and that VWF response to glucocorticoids is genetically determined and depends on the presence of particular polymorphisms in the VWF gene promoter. The risk of venous thromboembolism is moreover enhanced in patients with CS by additional endogenous and exogenous risk factors such as obesity, bed rest, surgery and invasive diagnostic procedures like inferior petrosal sinus (IPS) sampling. In line with all these data, patients with active CS should be treated as having a prothrombotic disorder and undergo antithrombotic prophylaxis during IPS sampling. Special care should be taken in the immediate perioperative period in order to avoid thromboembolic events. In the absence of prospective randomized trials, preventive antithrombotic treatment (best with heparin) during IPS sampling and low-dose heparin treatment early after surgery should be suggested.
库欣综合征(CS)患者存在高凝状态及其随之而来的血栓栓塞并发症发生率增加。这些改变与皮质醇过多有关,皮质醇通过多种复杂机制导致血液发生促血栓形成改变,包括凝血因子水平升高,主要是因子 VIII 和血管性血友病因子(VWF)以及纤溶能力受损。然而,最近观察到 VWF 水平的升高并非 CS 的固有特征,VWF 对糖皮质激素的反应是由遗传决定的,并且取决于 VWF 基因启动子中特定的多态性的存在。CS 患者由于肥胖、卧床休息、手术和侵袭性诊断程序(如蝶鞍旁窦(IPS)取样)等其他内源性和外源性危险因素,静脉血栓栓塞的风险进一步增加。根据所有这些数据,患有活动性 CS 的患者应被视为存在血栓形成倾向,并在 IPS 取样期间进行抗血栓预防。在围手术期应特别注意,以避免血栓栓塞事件。由于缺乏前瞻性随机试验,建议在 IPS 取样期间进行预防性抗血栓治疗(最好使用肝素),并在手术后早期进行低剂量肝素治疗。