Children's Hospital of Michigan, Wayne State University, Pediatric Hematology Oncology, Detroit, Michigan.
Children's Hospital of Michigan, Wayne State University, Detroit, Michigan.
Pediatr Blood Cancer. 2019 Jul;66(7):e27714. doi: 10.1002/pbc.27714. Epub 2019 Apr 4.
Von Willebrand disease (VWD) is an inherited bleeding disorder that is caused by a quantitative or qualitative deficiency of von Willebrand factor (VWF). The National Heart, Lung, and Blood Institute (NHLBI) guidelines for the diagnosis of VWD state that a VWF activity (VWF:RCo) of <30 IU/dL or <50 IU/dL with symptoms of clinical bleeding are consistent with the diagnosis of VWD. However, current gold-standard diagnostic testing takes days to have complete results. Thromboelastography (TEG) is a testing method that provides a graphical trace that represents the viscoelastic changes seen with fibrin polymerization in whole blood, therefore providing information on all phases of the coagulation process. This study describes the TEG characteristics in 160 patients who presented for workup of a bleeding disorder and a subset of those were subsequently diagnosed with VWD. The TEG parameters, K-time (representing the dynamics of clot formation) and the maximal rate of thrombus generation (MRTG), were found to be sensitive in detecting patients with VWF:RCo <30 IU/dL. The TEG, unlike VWF:RCo, can be done in real time, and results are available to the clinician within an hour. This will definitely be beneficial in acute situations such as evaluation of and management of acute bleeding in patients with acquired deficiencies of VWF and may play an important role in the surgical management of patients with VWD.
血管性血友病(VWD)是一种遗传性出血性疾病,由血管性血友病因子(VWF)的数量或质量缺乏引起。美国国立心肺血液研究所(NHLBI)的 VWD 诊断指南指出,VWF 活性(VWF:RCo)<30IU/dL 或有临床出血症状时<50IU/dL,与 VWD 的诊断一致。然而,目前的金标准诊断测试需要数天才能得出完整的结果。血栓弹力图(TEG)是一种测试方法,提供了一个图形轨迹,代表全血中纤维蛋白聚合时观察到的粘弹性变化,因此提供了关于凝血过程所有阶段的信息。本研究描述了 160 名因出血性疾病就诊并随后被诊断为 VWD 的患者的 TEG 特征。TEG 参数 K 时间(代表血凝块形成的动力学)和最大血栓生成速率(MRTG)被发现对 VWF:RCo <30IU/dL 的患者具有敏感性。TEG 与 VWF:RCo 不同,可实时进行,临床医生在一小时内即可获得结果。这在急性情况下肯定会有帮助,例如评估和管理 VWF 获得性缺乏患者的急性出血,并可能在 VWD 患者的手术管理中发挥重要作用。