ArticleHaemophilia : the official journal of the World Federation of Hemophilia
Delphi Consensus Recommendations for the Definition of a Severe Bleeding Phenotype and Initiation of Prophylaxis in Patients With Non-Severe Haemophilia.
Article in Haemophilia : the official journal of the World Federation of Hemophilia. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- When and How to Start Prophylaxis in Children with Hemophilia.Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie · 2026Review
- Focus on Women with Hemophilia and Carriers: How to Address All the Existing Gaps in Diagnosis and Care.Journal of clinical medicine · 2026Review
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11 authors.
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Abstract
introductionCurrent 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.
methodA 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.
resultsAfter 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.
conclusionConsensus was reached on criteria for SBPT and recommendations to initiate prophylaxis in patients with non-severe haemophilia that can be used in daily practice.
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