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

Authors: Pfrepper, C; Ay, C; Knöfler, R; Königs, C; Krause, M; Miesbach, W; Oldenburg, J; Sigl-Kraetzig, M; Alesci, RS; Olivieri, M

Affiliations: Division of Hemostaseology, Department of Hematology, Cellular Therapy, Hemostaseology and Infectiology, University Hospital Leipzig, Leipzig, Germany. Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria. Department of Pediatric Hemostaseology, Medical Faculty and University Hospital Carl Gustav Carus of Technische Universität Dresden, Dresden, Germany. Clinical and Molecular Hemostasis, Department of Pediatrics and Adolescent Medicine, Goethe University, University Hospital Frankfurt, Frankfurt, Germany. Department of Hemostaseology, Deutsche Klinik für Diagnostik HELIOS Klinik, Wiesbaden, Germany. Medical Clinic II, Goethe University, Frankfurt, Germany. Institute of Experimental Hematology and Transfusion Medicine, University Hospital Bonn, Medical Faculty, University of Bonn, Bonn, Germany. Institute For Pediatric Research and Further Education (IPFW), Blaubeuren, Germany. Pediatric Practice at the South Coagulation Center, Blaubeuren, Germany. MVZ IMD GmbH, IMD Blood Coagulation Centre Hochtaunus, Bad Homburg, Germany. Pediatric Thrombosis and Hemostasis Center, Pediatric Hemophilia Center, Dr. Von Hauner Children´S Hospital, LMU, Munich, Germany.

Publication: Haemophilia; 2026

ABSTRACT: 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.