Introduction: Patients with severe haemophilia and inhibitors against factor VIII who require surgery need a prophylactic approach to prevent bleeding complications. Scientific evidence to decide the best prophylactic treatment is very limited and mainly based on retrospective or case series.
Aims: To develop evidence- and expert opinion-based guidelines for prophylactic therapy for patients with haemophilia and inhibitors undergoing surgery.
Methods: A panel of nine Spanish haematologists undertook a systematic review of the literature and selected publications providing relevant information regarding the prophylactic management of patients with haemophilia and inhibitors undergoing dental extraction, minor surgery or major surgery.
Results: Although evidence is very limited, the panel considers that it seems advisable that prophylaxis should be given in most cases with a bypassing agent (aPCC or rFVIIa) and should start immediately before minor or major surgery. Patients should be closely monitored to enable dose/product modification as needed.
Conclusion: It is necessary to communicate clinical experience in a detailed way in order to ensure optimal schemes of prophylaxis for patients with haemophilia and inhibitors. Development of objective outcomes to evaluate efficacy is crucial.
Keywords: consensus; inhibitors; prophylaxis; surgery haemophilia.
© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.