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

Leukemia (2024)Cite this article

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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.

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Fig. 1: Baseline AVWS-related laboratories, multimer testing, clinical and long-term outcomes.

Data availability

Data available on request with email to corresponding author.

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Authors and Affiliations

  1. Department of Medicine, Mayo Clinic, Rochester, MN, USA

    Karan L. Chohan

  2. Division of Hematology, Mayo Clinic, Rochester, MN, USA

    Rajiv K. Pruthi, Saurabh Zanwar, Jonas Paludo, Ronald Go, Animesh Pardanani, Aneel Ashrani, Joselle M. Cook, Carrie A. Thompson, Thomas M. Habermann, Thomas E. Witzig, Morie A. Gertz, David Dingli, Fransis K. Buadi, Angela Dispenzieri, Nelson Leung, Shaji K. Kumar, Vincent Rajkumar, William L. Nichols, Robert A. Kyle, Stephen M. Ansell, Prashant Kapoor, Meera Sridharan & Jithma P. Abeykoon

  3. Division of Hematology, Mayo Clinic, Jacksonville, FL, USA

    Asher Chanan-Khan & Sikander Ailawadhi

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  1. Karan L. Chohan
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Contributions

KLC, RKP, MS, JPA designed this study, analyzed/interpreted the data and wrote the manuscript. SZ, JP, RG, AP, AA, JMC, CAT, ACK, SA, TMH, TEW, MAG, DD, FKB, AD, NL, SKK, WN, SMA, PK, interpreted the data and assisted in writing the manuscript. All authors provided final approval of the manuscript and are accountable for all aspects of the work.

Corresponding author

Correspondence to Jithma P. Abeykoon.

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The authors declare no competing interests.

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Chohan, K.L., Pruthi, R.K., Zanwar, S. et al. The clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events. Leukemia (2024). https://doi.org/10.1038/s41375-024-02393-6

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