The clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events

Abstract

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.