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    Poster Networking

    Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies

    Poster Networking Session

    PB0824 - Prospective longitudinal evaluation shows increase of VWF and FVIII activity with age in patients with type 1 and 2 Von Willebrand disease

    Monday, June 26, 2023
    18:30 – 19:30 ET
    Room: Exhibition

      Presenting Author(s)

    • Calvin B. van Kwawegen, MD (he/him/his)

      PhD-candidate
      Department of Hematology, Erasmus MC, Erasmus University Medical Center Rotterdam, Rotterdam, the Netherlands
      Rotterdam, Zuid-Holland, Netherlands

      • Co-Author(s)

    • Jeroen C.J Eikenboom, MD. PhD

      Professor, Head of Division of Thrombosis and Hemostasis
      Leiden University Medical Center
      Leiden, Zuid-Holland, Netherlands

    • PY

      Paula F. Ypma

      Hematologist
      Department Hematology, Hagaziekenhuis, Den Haag, the Netherlands
      Den Haag, Zuid-Holland, Netherlands

    • FH

      Floor C.J.I. Heubel-Moenen

      Hematologist
      Department Hematology, MUMC+, Maastricht, the Netherlands
      Maastricht, Limburg, Netherlands

    • Karin P. M. van Galen, MD PhD MsC

      Internist Hematologist
      Center for Benign Haematology, Thrombosis and Haemostasis, Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
      Utrecht, Utrecht, Netherlands

    • Karina Meijer, MD, PhD (she/her/hers)

      MD, PhD, Internist-haematologist, Professor
      Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
      Groningen, Groningen, Netherlands

    • SS

      Saskia E.M. Schols

      Internist-hematologist
      Department of Hematology, Radboud university medical center, Nijmegen, The Netherlands. Hemophilia Treatment Center Nijmegen – Eindhoven – Maastricht, The Netherlands
      Nijmegen, Gelderland, Netherlands

    • MC

      Marjon H. Cnossen

      Associate professor, pediatric hematologist, MD, PhD
      Department of Pediatric Hematology, Erasmus MC – Sophia Children’s Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands
      Rotterdam, Zuid-Holland, Netherlands

    • JB

      Johanna G. Bom, van der

      professor of Clinical Epidemiology
      Department Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands
      Leiden, Zuid-Holland, Netherlands

    • JM

      Joke Meris, de

      patient representative
      Netherlands Hemophilia Society, Nijkerk, the Netherlands
      Nijkerk, Gelderland, Netherlands

    • KF

      Karin Fijnvandraat

      Professor
      Amsterdam UMC, University of Amsterdam, Emma Children’s Hospital, Pediatric Hematology Amsterdam, Netherlands
      Amsterdam, Noord-Holland, Netherlands

    • Frank W.G. Leebeek, MD, PhD

      Professor and Head of Department of Hematology
      Erasmus MC, University Medical Center Rotterdam, The Netherlands
      Rotterdam, Zuid-Holland, Netherlands

    Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder with a heterogeneous phenotype. Cross-sectional studies suggest that Von Willebrand Factor activity (VWF-Act) and Factor VIII (FVIII:C) increase in VWD patients with aging.

    Aims: To evaluate VWF:Act and FVIII:C prospectively in a large cohort of VWD patients.

    Methods: VWF:Act and FVIII:C were measured in both the Willebrand in the Netherlands (WiN) study (between 2007-2009) and the Willebrand in the Netherlands Prospective Study (WiN Pro) (2019-2021) cohorts. VWD patients with data on VWF and FVIII activity from both studies were included in this preliminary analysis. VWF:Act was measured with the VWF:GPIbM Innovance assay from Siemens on a Sysmex CS-5100. FVIII:C was measured using a one‐stage clotting assay

    Results: In total, 202 VWD patients were included, of whom 116 type 1, 76 type 2 and 10 type 3 patients (Table 1). Among all participants, the VWF:Act and FVIII:C increased with a median of 0.08 (95%CI 0.06-0.10, p< 0.001) and 0.21 (95%CI 0.16-0.24, p< 0.001) U/mL over a period of approximately 12 years. For type 1 patients, the median increase of VWF:Act was 0.12 (95%CI 0.085-0.16) U/mL and the median increase of FVIII:C was 0.26 U/mL (95%CI 0.21-0.31; p< 0.001). For type 2 patients, the median increases of VWF:Act and FVIII:C were 0.05 (95%CI 0.025-0.070, p< 0.001) and 0.14 (95%CI 0.10-0.19, p< 0.001) U/mL. As expected, type 3 patients did not show a significant increase of VWF:Act or FVIII:C. The increase of VWF:Act and FVIII:C was found both in blood group O and non-O VWD patients.

    Conclusion(s): This first longitudinal prospective study in a well-defined cohort VWD patients shows an increase of VWF:Act and FVIII:C with age in both type 1 and type 2 VWD. Monitoring of the bleeding phenotype is ongoing to determine whether the observed increase has any effect on the bleeding phenotype.