Delphi Consensus Recommendations for the Definition of a Severe Bleeding Phenotype and Initiation of Prophylaxis in Patients With Non-Severe Haemophilia.

INTRODUCTION: Current guidelines recommend prophylaxis for patients with non-severe haemophilia with severe bleeding phenotype (SBPT) but there is no consensus how to define a SBPT and when to recommend prophylaxis in patients with non-severe haemophilia.

METHOD: A Delphi consensus procedure among the members of the Standing Committee Hemophilia of the German, Austrian and Swiss Society of Hemostasis and Thrombosis Research (GTH) was conducted. After defining 41 statements in a steering committee, 26 haemophilia experts participated. Statements were scored on a scale of 1-9, and agreement was defined as a score of ≥ 7. Consensus was defined as ≥ 75%, and strong consensus as ≥ 95% agreement.

RESULTS: After 3 rounds of consent, five major and three minor criteria for SBPT, five recommendations for starting long-term and six recommendations for starting intermittent prophylaxis were consented. Major criteria included life-threatening bleeding in critical regions or organs, severe bleeding that occurs spontaneously, repeatedly, or after inadequate trauma, development of haemophilic arthropathy, presence of chronic synovitis, and Hb-relevant menstrual bleeding. Long-term prophylaxis should be recommended in patients with a residual factor activity < 3 IU/dL, in patients with a residual activity > 3 IU/dL and a SBPT, following intracranial haemorrhage after assessing the individual risk of recurrence, in cases of comorbidities and medications that cause a permanently increased bleeding tendency, and in the presence of risk factors for severe bleeding or arthropathy.

CONCLUSION: Consensus was reached on criteria for SBPT and recommendations to initiate prophylaxis in patients with non-severe haemophilia that can be used in daily practice.

© 2026 The Author(s). Haemophilia published by John Wiley & Sons Ltd.

PMID: 41879621