Standardisation of Definition and Management for Bleeding Disorder of Unknown Cause: Communication from the SSC of the ISTH.

Baker, Ross I; Choi, Philip; Curry, Nicola; Gebhart, Johanna; Gomez, Keith; Henskens, Yvonne; Heubel-Moenen, Floor; James, Paula; Abdul Kadir, Rezan; Kouides, Peter; Lavin, Michelle; Lordkipanidze, Marie; Lowe, Gillian; Mumford, Andrew; Mutch, Nicola; Nagler, Michael; Othman, Maha; Pabinger, Ingrid; Sidonio, Robert; Thomas, Will; ... (2024). Standardisation of Definition and Management for Bleeding Disorder of Unknown Cause: Communication from the SSC of the ISTH. Journal of thrombosis and haemostasis, 22(7), pp. 2059-2070. Wiley-Blackwell 10.1016/j.jtha.2024.03.005

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In many patients referred with significant bleeding phenotype, laboratory testing fails to define any hemostatic abnormalities. Clinical practise with respect to diagnosis and management of this patient cohort poses significant clinical challenges. We recommend that bleeding history in these patients should be objectively assessed using the ISTH bleeding assessment tool (BAT). Patients with increased BAT scores should progress to hemostasis laboratory testing. To diagnose BDUC, normal complete blood count, prothrombin time, activated partial thromboplastin time, thrombin time, von Willebrand factor antigen, von Willebrand factor function; coagulation factors VIII, IX and XI and platelet light transmission aggregometry (LTA) should be the minimum laboratory assessment. In some laboratories, additional specialized haemostasis testing may be performed and identify other rare causes of bleeding. We recommend that patients with a significant bleeding phenotype but normal laboratory investigations should be registered with a diagnosis of 'Bleeding disorder of unknown cause (BDUC)' in preference to other terminology. Global haemostatic tests and markers of fibrinolysis demonstrate variable abnormalities, and their clinical significance remains uncertain. Targeted genomic sequencing examining candidate hemostatic genes has a low diagnostic yield. Underlying BDUC should be considered in patients with heavy menstrual bleeding since delays in diagnosis often extend to many years and negatively impact quality of life. Treatment options for BDUC patients include tranexamic acid, desmopressin and platelet transfusions.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Institute of Clinical Chemistry

UniBE Contributor:

Nagler, Michael

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1538-7836

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Pubmed Import

Date Deposited:

27 Mar 2024 12:11

Last Modified:

02 Jul 2024 00:13

Publisher DOI:

10.1016/j.jtha.2024.03.005

PubMed ID:

38518896

Uncontrolled Keywords:

heavy menstrual bleeding hemostatic platelet function tests tranexamic acid von Willebrand disease

BORIS DOI:

10.48350/194703

URI:

https://boris.unibe.ch/id/eprint/194703

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