{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T19:36:21Z","timestamp":1785267381705,"version":"3.55.0"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T00:00:00Z","timestamp":1774396800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T00:00:00Z","timestamp":1774396800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Current guidelines recommend prophylaxis for patients with non\u2010severe 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\u2010severe haemophilia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>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\u20139, and agreement was defined as a score of \u2265 7. Consensus was defined as \u2265 75%, and strong consensus as \u2265 95% agreement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>After 3 rounds of consent, five major and three minor criteria for SBPT, five recommendations for starting long\u2010term and six recommendations for starting intermittent prophylaxis were consented. Major criteria included life\u2010threatening 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\u2010relevant menstrual bleeding. Long\u2010term prophylaxis should be recommended in patients with a residual factor activity &lt; 3\u00a0IU\/dL, in patients with a residual activity &gt; 3\u00a0IU\/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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Consensus was reached on criteria for SBPT and recommendations to initiate prophylaxis in patients with non\u2010severe haemophilia that can be used in daily practice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70259","type":"journal-article","created":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T14:06:22Z","timestamp":1774447582000},"page":"665-670","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Delphi Consensus Recommendations for the Definition of a Severe Bleeding Phenotype and Initiation of Prophylaxis in Patients With Non\u2010Severe Haemophilia"],"prefix":"10.1111","volume":"32","author":[{"given":"Christian","family":"Pfrepper","sequence":"first","affiliation":[{"name":"Division of Hemostaseology Department of Hematology, Cellular Therapy, Hemostaseology and Infectiology University Hospital Leipzig  Leipzig Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cihan","family":"Ay","sequence":"additional","affiliation":[{"name":"Division of Hematology and Hemostaseology Department of Medicine I Medical University of Vienna  Vienna 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Thrombosis and Hemostasis Center Pediatric Hemophilia Center Dr. Von Hauner Children\u00b4S Hospital LMU  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the Standing Committee Hemophilia of the GTH","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,3,25]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02909.x"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2022007620"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15676"},{"key":"e_1_2_11_5_1","article-title":"Expert Opinion for Defining a Severe Bleeding Phenotype to Guide Prophylaxis in Patients With Nonsevere Hemophilia","author":"Pfrepper 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