Assessing the Bleeding Phenotype in Non-Severe hemophilia by use of the ISTH Bleeding Assessment Tool (BAT)

F.R. Kloosterman¹, A. Zwagemaker¹, C.N. Bagot², E.A.M. Beckers³, J.G. van der Bom⁴, P. Collins⁵, M.H. Cnossen⁶, M. Coppens⁷, S.C. Gouw^1,4, B.A.P. Laros-van Gorkum⁸, S. Mangles⁹, F.J.M. van der Meer¹⁰, K. Meijer¹¹, L. Nieuwenhuizen¹², R. Schutgens¹³, K. Fijnvandraat¹

¹Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Pediatric Hematology, Amsterdam, the Netherlands, ²NHS Greater Glasgow and Clyde, Department of Thrombosis and Haemostasis, Glasgow, United Kingdom, ³Maastricht University Medical Center (MUMC+), Hematology, Maastricht, the Netherlands, ⁴Leiden University Medical Center, Clinical Epidemiology, Leiden, the Netherlands, ⁵Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, United Kingdom, ⁶Erasmus University Medical Center - Sophia Children's Hospital, Department of Paediatric Hematology, Rotterdam, the Netherlands, ⁷Amsterdam UMC, University of Amsterdam, Vascular Medicine, Amsterdam, the Netherlands, ⁸Radboud university medical center, Hematology, Nijmegen, the Netherlands, ⁹Basingstoke and Hampshire Hospital, Hampshire, the Netherlands, ¹⁰Leiden Univeristy Medical Center, Department of Thrombosis and Haemostasis, Leiden, the Netherlands, ¹¹University Medical Center Groningen, Hematology, Groningen, the Netherlands, ¹²Maxima Medisch Centrum, Internal Medicine, Veldhoven, the Netherlands, ¹³Van Creveld Clinic, University Medical Center Utrecht, Utrecht, the Netherlands

Abstract Number: PB0897

Meeting: ISTH 2020 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Patients with non-severe hemophilia experience an increased bleeding tendency predominantly after provocative events such as trauma or surgery. Few studies have quantified the bleeding phenotype in this population. The International Society of Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) has been developed to objectively quantify bleeding symptoms and could provide insight into the extent of bleeding reported by non-severe hemophilia patients.

Aims: The aim of this study was to describe the phenotype of patients with non-severe hemophilia A and B using the patient reported ISTH-BAT.

Methods: The DYNAMO study is a cohort study that includes male congenital hemophilia A and B patients aged 12-55 years with a baseline clotting factor level of 2-35 IU/dL. ISTH-BAT scores were prospectively collected through an online questionnaire omitting gender-specific sections after written consent was obtained. The study was approved by the medical ethics committee (ClinicalTrials.gov Identifier: NCT03623295).

Results: In this preliminary analysis, 47/101 (47%) patients (37 HA and 10 HB) completed the questionnaire. The median age was 39 years (IQR 27-49) and the median FVIII/IX level was 12 IU/dL (IQR 6-17). We found a median ISTH-BAT score of 7 (IQR 3-13), with 74% reporting abnormal bleeding scores (≥4). Joint, muscle and mouth bleeds were reported most frequently (62, 45 and 45%) (Figure 1). Patients with moderate hemophilia (2-5 IU/dL) had higher ISTH-BAT scores compared to patients with mild hemophilia (5-35 IU/dL) (median ISTH-BAT score 10 vs 7). ISTH-BAT scores were similar between hemophilia A and B.

Conclusions: Patients with non-severe hemophilia A and B had a median ISTH-BAT of 7 with 74% of patients reporting abnormal bleeding scores. Patients with moderate hemophilia had higher ISTH-BAT-scores when compared to patients with mild hemophilia.

[Figure 1: Reported bleeding symptoms. Abbreviations; GI = gastro-intestinal, ICH = intracranial hemorrhage, Tooth Extr. = tooth extractions ]

To cite this abstract in AMA style:

Kloosterman FR, Zwagemaker A, Bagot CN, Beckers EAM, van der Bom JG, Collins P, Cnossen MH, Coppens M, Gouw SC, Laros-van Gorkum BAP, Mangles S, van der Meer FJM, Meijer K, Nieuwenhuizen L, Schutgens R, Fijnvandraat K. Assessing the Bleeding Phenotype in Non-Severe hemophilia by use of the ISTH Bleeding Assessment Tool (BAT) [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/assessing-the-bleeding-phenotype-in-non-severe-hemophilia-by-use-of-the-isth-bleeding-assessment-tool-bat/. Accessed May 6, 2021.

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