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Current practice and registration patterns among United Kingdom Haemophilia Centre Doctors' Organisation centers for patients with unclassified bleeding disorders.
- Source :
-
Journal of thrombosis and haemostasis : JTH [J Thromb Haemost] 2021 Nov; Vol. 19 (11), pp. 2738-2743. Date of Electronic Publication: 2021 Aug 25. - Publication Year :
- 2021
-
Abstract
- Background: Bleeding of unknown cause (BUC) and unclassified bleeding disorders (UBD) are increasingly recognized. There is no guidance on diagnosis and management.<br />Objectives: To examine UK haemophilia centre registration patterns and current practice for UBD patients.<br />Methods: In a two-step process, the UK National Haemophilia Database (NHD) was reviewed for registration patterns of UBD patients and a survey of UK haemophilia centers was conducted (January/February 2021) to capture current practice for diagnosis and management of patients with UBD.<br />Results/discussion: Overall, registrations with the NHD for UBD patients has sharply risen from 2012 to 2020 and in 2019 accounted for 2.65% of registered patients. For the survey, the response rate was 52/67 (78%). Practice was widely variable; 35/52 (67%) centers register UBD; among these 35 centers, terminology included UBD (28 centers), undiagnosed bleeding disorder (four centers), and BUC (three centers); 34/52 (65%) centers use a formal bleeding assessment tool. For management of dental extraction and high bleeding risk surgery in a fictional UBD patient we found that tranexamic acid was widely used; however, beyond this a variety of hemostatic products were advised including blood products, recombinant factor VIIa/prothrombin complex concentrate, and desmopressin. There was general consensus (≈90%) on avoiding regional anesthesia in pregnancy, but no agreement on the need for fetal precautions to avoid bleeding at delivery (50% would advise these). There was a disparity of opinion on chemical thromboprophylaxis, and management of patients without prior hemostatic challenges and offspring of these patients.<br />Conclusion: This study provides a snapshot of current practice and real-world data in this area. Future studies need to address the gaps in evidence.<br /> (© 2021 International Society on Thrombosis and Haemostasis.)
Details
- Language :
- English
- ISSN :
- 1538-7836
- Volume :
- 19
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Journal of thrombosis and haemostasis : JTH
- Publication Type :
- Academic Journal
- Accession number :
- 34382329
- Full Text :
- https://doi.org/10.1111/jth.15492