An under-recognized source of bleeding in Waldenström macroglobulinemia (WM) is the development of an acquired von Willebrand syndrome (AVWS) [1]. In WM, the von Willebrand Factor (vWF) glycoprotein can be degraded due to autoantibody destruction, increased shear stress due to hyperviscosity, or sequestered due to adsorption onto malignant cells, leading to the development of AVWS (AVWS-WM) [1, 2]. However, there is currently a paucity of published literature characterizing the clinical features associated with AVWS-WM and, specifically, the change in bleeding-related symptoms through treatment [3,4,5,6]. In this study, we assessed the prevalence of AVWS-WM in patients with WM treated in a tertiary care center and evaluated the associated clinical manifestations and outcomes of patients with AVWS-WM compared to those with WM and without AVWS.

This retrospective cohort study evaluated patients with active WM seen at Mayo Clinic and affiliated practices from January 2002 to January 2022. Patients were identified using the Mayo Clinic Data Explorer, and those who underwent vWF testing and had confirmed a diagnoses of WM and AVWS were included [7]. A matched control cohort (patients with WM and without AVWS) with a 5:1 ratio based on age, sex, and diagnosis date was also established. Detailed methodologies are available in the Supplementary Materials.

华氏巨球蛋白血症 （WM） 中一个未被充分认识的出血来源是获得性血管性血友病综合征 （AVWS） 的发生[1]。在 WM 中，血管性血友病因子 （vWF） 糖蛋白可因自身抗体破坏而降解，因高粘度而增加剪切应力，或因吸附到恶性细胞上而被隔离，从而导致 AVWS （AVWS-WM） 的发展 [1， 2]。然而，目前缺乏已发表的文献来描述与 AVWS-WM 相关的临床特征，特别是治疗后出血相关症状的变化 [3,4,5,6]。在这项研究中，我们评估了在三级医疗中心接受治疗的 WM 患者中 AVWS-WM 的患病率，并评估了 AVWS-WM 患者与有 WM 和无 AVWS 患者相比的相关临床表现和结果。

这项回顾性队列研究评估了 2002 年 1 月至 2022 年 1 月期间在妙佑医疗国际及其附属诊所就诊的活动性 WM 患者。使用 Mayo Clinic Data Explorer 确定患者，包括接受 vWF 检测并确诊 WM 和 AVWS 的患者 [7]。还建立了一个匹配的对照组 （WM 和无 AVWS 患者），根据年龄、性别和诊断日期的比例为 5：1。补充材料中提供了详细的方法。