Management of rare inherited bleeding disorders: Proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders.
acute haemorrhage
coagulation factor deficiency
pregnancy
prophylaxis
rare bleeding disorders
surgery
Journal
European journal of haematology
ISSN: 1600-0609
Titre abrégé: Eur J Haematol
Pays: England
ID NLM: 8703985
Informations de publication
Date de publication:
Jun 2023
Jun 2023
Historique:
revised:
25
01
2023
received:
20
10
2022
accepted:
31
01
2023
medline:
4
5
2023
pubmed:
8
2
2023
entrez:
7
2
2023
Statut:
ppublish
Résumé
The rare coagulation disorders may present significant difficulties in diagnosis and management. In addition, considerable inter-individual variation in bleeding phenotype is observed amongst affected individuals, making the bleeding risk difficult to assess in affected individuals. The last international recommendations on rare inherited bleeding disorders (RIBDs) were published by the United Kingdom Haemophilia Centre Doctors' Organisation in 2014. Since then, new drugs have been marketed, news studies on surgery management in patients with RIBD have been published, and new orphan diseases have been described. Therefore, the two main objectives of this review, based on the recent recommendations published by the French Reference Centre on Haemophilia and Rare Bleeding Disorders, are: (i) to briefly describe RIBD (clinical presentation and diagnostic work-up) to help physicians in patient screening for the early detection of such disorders; and (ii) to focus on the current management of acute haemorrhages and long term prophylaxis, surgical interventions, and pregnancy/delivery in patients with RIBD.
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
584-601Informations de copyright
© 2023 The Authors. European Journal of Haematology published by John Wiley & Sons Ltd.
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