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    Poster Networking

    Hemophilia and Rare Bleeding Disorders

    Poster Networking Session

    PB0160 - The impact of past inhibitor on health status in patients with haemophilia a in The Netherlands

    Sunday, June 25, 2023
    18:30 – 19:30 ET
    Room: Exhibition

      Presenting Author(s)

    • Letícia Lemos Jardim, PhD (she/her/hers)

      Researcher
      Instituto René Rachou (Fiocruz Minas), Belo Horizonte, Minas Gerais, Brazil
      Belo Horizonte, Minas Gerais, Brazil

      • Co-Author(s)

    • JB

      Johanna G. Bom, van der

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

    • Suely Rezende, MD, PhD (she/her/hers)

      Professor of Internal Medicine
      Universidade Federal de Minas Gerais, Brazil

    • Lv

      Lize F.D. van Vulpen

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

    • Jeroen C.J Eikenboom, MD. PhD

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

    • EB

      Erik A.M. Beckers

      Head of Haematology
      Department of Hematology, Maastricht University Medical Center + and Maastricht University, Maastricht, The Netherlands
      Maastricht, Limburg, Netherlands

    • LH

      Louise Hooijmeijer

      Hematologist
      Division of Hematology/Oncology, Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
      Groningen, Groningen, Netherlands

    • Michiel Coppens, MD, PhD (he/him/his)

      Medical Specialist
      Amsterdam University Medical Centers, Amsterdam, The Netherlands
      Amsterdam, Noord-Holland, 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

    • 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

    • MD

      Mariëtte Driessens

      Dutch Association of Hemophilia Patients
      Dutch Association of Hemophilia Patients
      Leiden, Zuid-Holland, Netherlands

    • FR

      Frits R. R. Rosendaal

      Professor
      Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands
      Leiden, Zuid-Holland, Netherlands

    • Samantha Gouw, MD, PhD (she/her/hers)

      Pediatric Hematologist
      The University of Amsterdam
      Amsterdam, Noord-Holland, Netherlands

    Background: With the emergence of prophylaxis and new therapies, treatment of haemophilia A(HA) has improved, especially for patients with inhibitors.

    Aims: To describe the impact of past inhibitors on joint health and activity of patients with HA(PwHA).

    Methods: This cross-sectional study analysed data from the sixth Haemophilia in the Netherlands (HiN6), conducted during 2018–2019. We evaluated the questionnaires including the Haemophilia Activities List(HAL) and the medical records of Dutch males with HA. Patients were stratified by age, HA severity, history of inhibitor and inhibitor titre.

    Results: In total, 685 PwHA were included [median age 45 years (IQR22-60)], of whom 28(41%) had severe HA(FVIII:C < 1%). History of inhibitor development was reported by 58 severe patients and 40 non-severe. Of the 98 patients with inhibitor, 25(25%) had a current inhibitor. A total of 8% had high-titre inhibitors and 55/98(56%) underwent immune tolerance induction (ITI). The frequency of reported chronic joint problems due to haemophilia, pain intensity and the frequency of hospitalization for orthopaedic surgery did not differ between patients with and without history of inhibitors independent of the severity. Limitations for lifting, climbing stairs, kneeling, walking and selfcare increased with age, but did not differ with history of inhibitors in patients < 60 years, even when stratified by titre. Yet, the differences between with/without inhibitors were significant (p < 0.05) in severe patients >60 years (Figure1). Overall, HAL scores for non-severe PwHA were higher in comparison with severe HA, but the score did not differ by history of past inhibitors, independent of severity.

    Conclusion(s): History of inhibitor was not associated with worse joint health or activity/participation level in PwHA in The Netherlands. Although joint problems continue to be frequently reported in elderly PwHA, we did not observe differences between persons with or without inhibitors. It will be interesting to compare these data after the introduction of Emicizumab.