High Molecular Weight von Willebrand Factor Multimer Loss and Bleeding in Patients with Short-Term Mechanical Circulatory Support Devices: A Case Series.
High Molecular Weight von Willebrand Factor Multimer Loss and Bleeding in Patients with Short-Term Mechanical Circulatory Support Devices: A Case Series.
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使用短期机械循环支持装置的患者的高分子量冯维勒布兰德因子多聚体丢失和出血：病例系列。
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发表时间：
2018
期刊：
影响因子：
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通讯作者：
O. Volod
中科院分区：
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作者：
M. Goldfarb;L. Czer;L. Lam;J. Moriguchi;F. Arabía;O. Volod
由于高分子量多聚体（HMWM）丢失导致的获得性von Willebrand综合征（VWS）已报告使用长期机械器械，并与粘膜出血（一种主要止血类型）相关。然而，很少有人知道类似的缺陷是否发生在短期机械循环支持（STMCS）设备的患者。我们回顾了2015年12月至2017年3月在一家学术四级护理医院接受VWS检查的STMCS器械患者的血管性血友病因子（VWF）特征。共有18例患者（57.0 ± 12.7岁; 83.3%为男性），包括9例粘膜出血和9例血红蛋白降低。STMCS器械包括Impella（n = 11）、Impella和右心室辅助器械（n = 2）和体外膜式氧合器（n = 5）。通过定量VWF多聚体分析的平均HMWM为3.6% ± 1.3%（正常临界值：18-34%）。在获得VWF活性、纤维蛋白原、因子VIII或VWF抗原水平的所有10例病例中，它们均正常或升高。所有病例均表现出正常或升高水平的低分子量多聚体（LMWMs）。这些结果与2型VWS（质量缺陷）一致。这是第一项定量描述STMCS器械相关HMWM损失的研究，HMWM损失可能导致粘膜出血。这一发现可能对STMCS期间植入长期器械或心脏移植或其他手术的术中管理有影响。
Acquired von Willebrand syndrome (VWS) due to loss of high-molecular-weight multimers (HMWMs) has been reported with longer term mechanical devices and is associated with mucosal bleeding, a primary hemostasis type of bleeding. However, little is known whether a similar defect occurs in patients with short-term mechanical circulatory support (STMCS) devices. We reviewed von Willebrand factor (VWF) profiles in patients with STMCS devices who underwent VWS workup from December 2015 to March 2017 at an academic quaternary care hospital. There were a total of 18 patients (57.0 ± 12.7 years old; 83.3% male) including nine with mucosal bleeding and nine with decreasing hemoglobin. The STMCS devices included Impella (n = 11), Impella and right ventricular assist device (n = 2), and an extracorporeal membrane oxygenator (n = 5). The mean HMWM by quantitative VWF multimer analysis was 3.6% ± 1.3% (normal cutoff: 18-34%). In all 10 cases in which VWF activity, fibrinogen, factor VIII, or VWF antigen level were obtained, they were either normal or elevated. All cases demonstrated high normal or elevated levels of low molecular weight multimers (LMWMs). These findings are consistent with type 2 VWS (qualitative defect). This is the first study that quantitatively describes STMCS device-associated HMWM loss, which may contribute to mucosal bleeding. This finding may have implications for intraoperative management during implantation of longer term devices or heart transplantation or other surgery while on STMCS.