Management of congenital quantitative fibrinogen disorders: a Delphi consensus. (19th September 2016)

• Record Type: Journal Article
• Authors: Casini, A.; de Moerloose, P.
• Other Names: Barbay Virginie investigator.; Barro Claire investigator.; Biron Christine investigator.; Boehlen Françoise investigator.; Brand Brigit investigator.; Brungs Thomas investigator.; Chamouni Pierre investigator.; Claeyssens Ségolène investigator.; Colin Véronique investigator.; de Maistre Emmanuel investigator.; Desconclois Céline investigator.; Devalet Bérangère investigator.; D'Oiron Roseline investigator.; Fontana Pierre investigator.; Hanss Michel investigator.; Hermans Cédric investigator.; Lavenu‐Bombled Cécile investigator.; Lebreton Aurélien investigator.; Mouton Christine investigator.; Noetzli Jasmine investigator.
• Is Part Of: Haemophilia. Volume 22:Number 6(2016:Nov.)
• Page Start: 898
• Page End: 905
• Publication Date: 2016-09-19
• Subjects: afibrinogenaemia -- bleeding -- fibrinogen -- hypofibrinogenaemia -- pregnancy -- thrombosis; Hemophilia -- Periodicals; 616.1572005
• DOI: 10.1111/hae.13061
• Languages: English
• ISSNs: 1351-8216

Abstract

Abstract : Introduction: No evidence‐based guidelines for the management of patients suffering from afibrinogenaemia and hypofibrinogenaemia are available. Aim and method: The aim of this study was to harmonize patient's care among invited haemophilia experts from Belgium, France and Switzerland. A Delphi‐like methodology was used to reach a consensus on: prophylaxis, bleeding, surgery, pregnancy and thrombosis management. Results: The main final statements are as follows: (i) a secondary fibrinogen prophylaxis should be started after a first life‐threatening bleeding in patients with afibrinogenaemia; (ii) during prophylaxis the target trough fibrinogen level should be 0.5 g L −1 ; (iii) if an adaptation of dosage is required, the frequency of infusions rather than the fibrinogen amount should be modified; (iv) afibrinogenaemic patients undergoing a surgery at high bleeding risk should receive fibrinogen concentrates regardless of the personal or family history of bleeding; (v) moderate hypofibrinogenaemic patients (i.e. ≥0.5 g L −1 ) without previous bleeding (despite haemostatic challenges) undergoing a surgery at low bleeding risk may not receive fibrinogen concentrates as prophylaxis; (vi) monitoring the trough fibrinogen levels should be performed at least once a month throughout the pregnancy and a foetal growth and placenta development close monitoring by ultrasound is recommended; (vii) fibrinogen replacement should be started concomitantly to the introduction of anticoagulation in afibrinogenaemic patients suffering from a venous thromboembolic event; and (viii) low‐molecular‐weight heparin is the anticoagulant of choice in case of venous thromboembolism. Conclusion: The results of this initiative should help clinicians in the difficult management of patients with congenital fibrinogen disorders.