Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy
Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy
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DOI：
10.5551/jat.27268
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发表时间：
2015-01-01
影响因子：
4.4
通讯作者：
Zieger, Barbara
中科院分区：
文献类型：
--
作者：
Kalbhenn, Johannes;Schmidt, Rene;Zieger, Barbara
目的：获得性血管性血友病综合征（AVWS）是一种获得性出血性疾病，据报道可加重心室辅助装置或主动脉瓣狭窄患者的出血并发症。AVWS的特征在于血管性血友病因子（VWF）的高分子量（HMW）多聚体的损失，从而损害VWF与血小板和胶原的结合。本研究的目的是调查AVWS的发展与静脉静脉ECMO（体外膜肺氧合）support.Methods治疗的患者：我们研究了AVWS的存在，在成人患者接受ECMO支持（n=18）和对照组治疗无ECMO支持（n=18）。AVWS的诊断基于胶原结合能力与VWF抗原的比率（VWF：CB/VWF：Ag）和VWF多聚体分析。结果：所有患者在ECMO支持下均发生AVWS。AVWS在ECMO植入后早期即ECMO植入后24小时内发现。在17例患者中，VWF：CB/VWF：Ag比值显著降低，HMW多聚体严重缺失，18例患者中有17例发生出血并发症，需要输血、FFP和/或血小板浓缩物。此外，9例患者没有ECMO设备进行了调查（ECMO植入前：n=2，ECMO植入后：n=7）。结论：在这项研究中，所有患者与ECMO支持治疗发展AVWS，AVWS是可检测的ECMO植入后24小时内。ECMO复律后AVWS可逆转。早期诊断AVWS并提供适当的治疗可能会降低危及生命的出血的发生率。
Aims: Acquired Von Willebrand syndrome (AVWS) is an acquired bleeding disorder that has been reported to aggravate bleeding complications in patients with ventricular assist devices or aortic stenosis. AVWS is characterized by the loss of the high-molecular-weight (HMW) multimers of Von Willebrand factor (VWF) with consequent impaired VWF binding to platelets and collagen. The aim of this study was to investigate the development of AVWS in patients treated with veno-venous ECMO (extracorporeal membrane oxygenation) support.Methods: We examined the presence of AVWS in adult patients receiving ECMO support (n=18) and control subjects treated without ECMO support (n=18). The diagnosis of AVWS was made based on the ratio of collagen-binding capacity to VWF-antigen (VWF: CB/VWF:Ag) and a VWF multimeric analysis. In addition, bleeding episodes were monitored.Results: All patients supported with ECMO developed AVWS. AVWS was identified in the early period after ECMO implantation, i.e. within 24 hours after ECMO implantation. In 17 patients, the VWF: CB/VWF: Ag ratio was significantly reduced and HMW multimers were severely missing, and 17 of the 18 patients developed bleeding complications and required transfusions of blood, FFP and/or platelet concentrates. In addition, nine patients without an ECMO device were investigated (prior to ECMO implantation: n=2, after ECMO explantation: n=7).Conclusions: In this study, all patients treated with ECMO support developed AVWS, and AVWS was detectable within 24 hours after ECMO implantation. However, the AVWS was reversible after ECMO explantation. Making an early diagnosis of AVWS and providing appropriate treatment may reduce the incidence of life-threatening bleeding.