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    Poster Networking

    Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies

    Poster Networking Session

    PB0829 - Defining the Impact of Ageing on ISTH-BAT (ISTH-SSC endorsed Bleeding Assessment Tool) Scores in Women With Low von Willebrand Factor (VWF) Levels

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

      Presenting Author(s)

    • Dearbhla Doherty, MD (she/her/hers)

      Clinical Research Fellow
      Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland
      Dublin 2, Dublin, Ireland

      • Co-Author(s)

    • Pamela A. Christopherson

      Research Program Coordinator
      Versiti Blood Research Institute
      Milwaukee, Wisconsin, United States

    • MB

      Mary Byrne

      Coagulation Laboratory Chief
      National Coagulation Centre, St James's Hospital, Dublin
      Dublin, Dublin, Ireland

    • MN

      Margaret Nolan

      Clinical Research Coordinator
      National Coagulation Centre, St James's Hospital, Dublin 8
      Dublin, Dublin, Ireland

    • Jamie O'Sullivan, PhD (she/her/hers)

      Principal Investigator
      Royal College of Surgeons in Ireland, Irish Centre for Vascular Biology, Dublin, Ireland

    • KR

      Kevin Ryan

      Consultant Haematologist
      National Coagulation Centre, St James's Hospital, Dublin
      Dublin, Dublin, Ireland

    • NO

      Niamh O'Connell, MD

      Consultant Haematologist
      National Coagulation Centre, St James's Hospital, Dublin
      Dublin, Dublin, Ireland

    • RM

      Robert Montgomery

      Senior Investigator
      Medical College of Wisconsin, Versiti Blood Research Institute, Children's Research Institute
      Milwaukee, Wisconsin, United States

    • Veronica H. Flood, MD (she/her/hers)

      Professor
      Medical College of Wisconsin
      Milwaukee, Wisconsin, United States

    • Sandra Haberichter, PhD

      Director, Hemostasis, Senior Investigator
      Versiti Blood Research Institute
      Milwaukee, Wisconsin, United States

    • Jorge Di Paola, MD (he/him/his)

      Professor of Pediatrics and Molecular Genetics & Genomics
      Washington University School of Medicine, United States

    • Paula James, MD

      Professor
      Department of Medicine, Queen's University, Kingston, ON, Canada
      Kingston, Ontario, Canada

    • Michelle Lavin, MB, PhD (she/her/hers)

      Consultant Haematologist
      National Coagulation Centre, St. James Hospital and Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Physicians and Surgeons in Ireland (RCSI)
      Dublin, Dublin, Ireland

    • JO

      James S. O'Donnell, MB, PhD

      Professor of Haematology
      Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland,Dublin, Ireland
      Dublin, Dublin, Ireland

    Background: ASH/ISTH/NHF/WFH guidelines recommend the use of Bleeding Scores (BS) to evaluate bleeding phenotype severity in VWD. Importantly, ageing has been shown to impact BS in patients with severe type 1 VWD and BS>10 have been shown to predict future bleeding risk. However, the clinical utility of BS and effects of ageing have not been determined for patients with Low VWF (VWF:Ag 30-50IU/dL).

    Aims: To investigate the clinical utility and impact of ageing on ISTH-BAT scores in women with Low VWF.

    Methods: BS data from two cohorts of well-characterized patients (Lovic Study, Dublin, Ireland and Zimmerman Program, Milwaukee, USA) were evaluated. Females ≥18 years with Low VWF levels and significant bleeding phenotype (diagnostic ISTH-BAT≥6) were included.

    Results: Ageing was associated with ~0.9 unit increase in diagnostic ISTH-BAT per decade (β=0.86, 95%CI=0.26-1.45, n=145, age range 18-66). Critically, ageing was also associated with an increased likelihood of ISTH-BAT>10 (OR=1.38/decade, 95%CI=1.02-1.88). Overall ISTH-BAT scores at time of diagnosis were significantly higher in older women (Oldest quartile (n=37)-vs-Youngest quartile (n=39), Median 11-vs-8, p=0.0079, Table.1). On sub-domain analyses, challenge scores were increased in older women, due to higher rates of post-surgical bleeding (OR=2.99, 95%CI=1.19-7.28, Fig.1/Table.1), secondary to increased exposure to surgical challenges (prior surgery; 86-vs-57%, p=0.02). Similarly, sex-specific scores were increased, with higher rates of delivery (80%-vs-17%, p< 0.0001) and postpartum haemorrhage (OR=9.14, 95%CI=2.54-31.43). Furthermore, maximal menorrhagia scores (score of 4; OR=10.29, 95%CI=2.94-30.28) were markedly more frequent in older women, likely due to increasing acceptability of surgical management of menorrhagia with ageing. In contrast, there was no increase in spontaneous scores with ageing, suggesting no difference in true bleeding phenotype.

    Conclusion(s): Age-related increases in ISTH-BAT in women with Low VWF are due to accrual of haemostatic challenges, as well as higher menorrhagia domain scores. Our data highlight the need to account for age when utilising ISTH-BAT to define bleeding severity.