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Journal article
Bleeding Disorder of Unknown Cause (BDUC): an illustrated review on current practice, knowledge gaps and future perspectives
Research and Practice in Thrombosis and Haemostasis, Vol.8(8), 102625
2024
Abstract
In more than half of the individuals with a clinically relevant bleeding tendency who are referred to hemostasis experts, no biological etiology can be found after extensive laboratory testing. These persons are diagnosed with an unexplained bleeding tendency or ‘bleeding disorder of unknown cause’ (BDUC). The mucocutaneous bleeding phenotype of individuals with BDUC is generally comparable to that of individuals with inherited bleeding disorders such as von Willebrand disease or platelet function disorders. BDUC definitions applied in literature are heterogeneous, but all comprise two main criteria: (1) there is an increased bleeding tendency based on the clinical view of the physician and/or an increased bleeding score; (2) no abnormalities are found with available hemostasis laboratory tests. This is reflected in the recent published BDUC definition by the ISTH SSC, stating that BDUC is a diagnosis of exclusion, characterized by normal hemostatic investigations despite a clinically significant bleeding tendency. Importantly, other non-hemostatic and acquired causes of bleeding should be excluded, but details on exclusion criteria and associated diagnostic testing remain undefined. Patients and healthcare providers are challenged by the uncertainty and lack of formal diagnosis particularly as there is no clear consensus regarding treatment. Research on the diagnostic value of new laboratory tests in individuals with BDUC has not yet been productive. In this illustrative review, the current practice and knowledge gaps in BDUC are addressed, previous research on BDUC is outlined and future directions with outstanding questions for future research in BDUC are highlighted.
Details
- Title
- Bleeding Disorder of Unknown Cause (BDUC): an illustrated review on current practice, knowledge gaps and future perspectives
- Authors/Creators
- Amaury L.L. MonardCaroline M.A. MussertTirsa T. DuijlMarieke J.H.A. KruipYvonne HenskensMaarrtje van den BiggelaarRoger E.G. SchutgensSaskia E M Schols - Radboud University NijmegenKarin J. FijnvandraatKarina Meijer - University Medical Center GroningenPaul L. den ExterLaurens NieuwenhuizenIris van MoortRoss Baker - Murdoch University, Centre for Molecular Medicine and Innovative TherapeuticsJames S. O’Donnell - St. James's HospitalMarjon H Cnossen - Erasmus MC - Sophia Children’s HospitalFloor C J I Heubel-Moenen - Maastricht University
- Publication Details
- Research and Practice in Thrombosis and Haemostasis, Vol.8(8), 102625
- Publisher
- Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.
- Identifiers
- 991005715169607891
- Copyright
- © 2024 The Authors.
- Murdoch Affiliation
- Murdoch University; Centre for Molecular Medicine and Innovative Therapeutics
- Resource Type
- Journal article
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