Treatment of patients with rare bleeding disorders in the Netherlands: Real-life data from the RBiN study.

Autor: Maas DPMSM; Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.; Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, the Netherlands., Saes JL; Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.; Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, the Netherlands., Blijlevens NMA; Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands., Cnossen MH; Department of Pediatric Hematology and Oncology, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, the Netherlands., den Exter PL; Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, the Netherlands., Kruis IC; Netherlands Hemophilia Society, Nijkerk, the Netherlands., Meijer K; Department of Hematology, University Medical Center Groningen, Groningen, the Netherlands., Nieuwenhuizen L; Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, the Netherlands.; Department of Hematology, Maxima Medical Center, Eindhoven, the Netherlands., Peters M; Department of Pediatric Hematology, Amsterdam University Medical Centers, Location Emma Children's Hospital, Amsterdam, the Netherlands., Schutgens REG; Department of Benign Hematology, van Creveldkliniek, University Medical Center Utrecht and University Utrecht, Utrecht, the Netherlands., van Heerde WL; Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.; Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, the Netherlands.; Enzyre BV, Novio Tech Campus, Nijmegen, the Netherlands., Schols SEM; Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.; Hemophilia Treatment Center, Nijmegen-Eindhoven-Maastricht, the Netherlands.
Jazyk: angličtina
Zdroj: Journal of thrombosis and haemostasis : JTH [J Thromb Haemost] 2022 Apr; Vol. 20 (4), pp. 833-844. Date of Electronic Publication: 2022 Feb 08.
DOI: 10.1111/jth.15652
Abstrakt: Background: Patients with rare inherited bleeding disorders (RBDs) exhibit hemorrhagic symptoms, varying in type and severity, often requiring only on-demand treatment. Prolonged bleeding after invasive procedures is common. Adequate peri-procedural therapy may reduce this bleeding risk.
Objective: To describe general treatment plans of RBD patients and evaluate the use of peri-procedural hemostatic therapy.
Methods: In the Rare Bleeding Disorders in the Netherlands (RBiN) study, RBD patients from all six Dutch Hemophilia Treatment Centers were included. General treatment plans were extracted from patient files. Patients with a dental or surgical procedure in their history were interviewed about use of peri-procedural treatment and bleeding complications.
Results: Two-hundred sixty-three patients with a rare coagulation factor deficiency or fibrinolytic disorder were included. Eighty-four percent had a documented general treatment plan. General treatment plans of patients with the same RBD were heterogeneous, particularly in factor XI deficiency. Overall, 308 dental and 408 surgical procedures were reported. Bleeding occurred in 50% of dental and 53% of surgical procedures performed without hemostatic treatment and in 28% of dental and 19% of surgical procedures performed with hemostatic treatment. Not only patients with severe RBDs, but also patients with mild deficiencies, experienced increased bleeding without proper hemostatic treatment.
Conclusion: Large heterogeneity in general treatment plans of RBD patients was found. Bleeding after invasive procedures was reported frequently, both before and after RBD diagnosis, irrespective of factor activity levels and particularly when peri-procedural treatment was omitted. Improved guidelines should include uniform recommendations for most appropriate hemostatic products per RBD and emphasize the relevance of individual bleeding history.
(© 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.)
Databáze: MEDLINE
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