Delphi consensus recommendations for the definition of a severe bleeding phenotype and initiation of prophylaxis in patients with non-severe hemophilia

Introduction Current guidelines recommend prophylaxis for patients with non-severe hemophilia with severe bleeding phenotype (SBPT). However, there is no consensus how to define a SBPT and thus when to recommend prophylaxis in patients with non-severe hemophilia. Method Members of the Standing Committee Hemophilia of the German, Austrian and Swiss Society of Hemostasis and Thrombosis Research (GTH) were asked to participate in a Delphi consensus procedure for the definition of a SBPT and the recommendation of prophylaxis in patients with non-severe hemophilia. After defining 41 statements in a steering committee, 26 hemophilia experts participated in the Delphi process. Statements were scored on a scale of 1 to 9, and agreement was defined as a score of≥7. Consensus was defined as≥75% agreement among participants, and strong consensus as≥95% agreement. Results After 3 rounds of consent, five major criteria and three minor criteria for SBPT were consented. Major criteria included life-threatening bleeds in critical regions or organs that occurred spontaneously or after minor trauma, severe bleeding that occurs spontaneously, repeatedly, or after inadequate trauma, such as muscle bleeding or bleeding with consequences for function and structure, development of hemophilic arthropathy, presence of chronic synovitis, and Hb-relevant menstrual bleeding despite exhausting conservative measures such as tranexamic acid, DDAVP, and/or hormone therapy. In addition, five recommendations for starting continuous and six recommendations for starting intermittent prophylaxis were consented. Continuous prophylaxis should be recommended in patients with a residual factor activity3% and a SBPT, following intracranial hemorrhage 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 major and minor criteria for SBPT, along with specific recommendations for when to initiate prophylaxis in patients with bleeding disorders. These consensus recommendations can be used in daily practice to assist clinicians in deciding when to recommend prophylaxis in patients with non-severe hemophilia. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany