{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:48:54Z","timestamp":1789663734797,"version":"4.0.1"},"reference-count":35,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,3,6]],"date-time":"2024-03-06T00:00:00Z","timestamp":1709683200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Extended half-life (EHL) factor IX (FIX) concentrates allow for prophylaxis with prolonged dosing intervals and high bleeding protection in persons with hemophilia B. Long-term real-world studies are lacking. Methods: In a retrospective\u2013prospective study, the six-year use of prophylaxis with the EHL recombinant FIX\u2013albumin fusion protein (rIX-FP) was analyzed, comparing outcomes with previous standard half-life (SHL) FIX in patients already on prophylaxis. Results: Prophylaxis with rIX-FP was prescribed in 15 patients (10 severe, 5 moderate; follow-up: 57 \u00b1 17 months). Based on a pharmacokinetic assessment and clinical needs, the first regimen was 47 \u00b1 7 IU\/Kg every 9 \u00b1 2 days. All but one patient remained on rIX-FP prophylaxis, adjusting infusion frequency and\/or dose; the last prescribed frequency was \u226510 days in 10\/13 patients, being reduced in seven and increased in four vs. the first regimen. The weekly FIX dose was unchanged; FIX trough levels were &gt;5% in all patients. The annual infusion number and FIX IU\/Kg significantly decreased (~60%) in eight patients previously on SHL FIX prophylaxis, with similar concentrate costs. Very low bleeding rates (most traumatic bleeds and the last quartile of the infusion interval), improved orthopedic and pain scores, unchanged HEAD-US scores and problem joints, and high treatment adherence (&gt;90%) and satisfaction were registered. Conclusions: Personalized, carefully adjusted rIX-FP regimens contribute to the diffusion and optimization of prophylaxis in persons with severe and moderate hemophilia B, with long-term favorable bleeding, joint, and patient-reported outcomes.<\/jats:p>","DOI":"10.3390\/jcm13051518","type":"journal-article","created":{"date-parts":[[2024,3,6]],"date-time":"2024-03-06T08:43:14Z","timestamp":1709714594000},"page":"1518","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Six-Year, Real-World Use of Prophylaxis with Recombinant Factor IX\u2013Albumin Fusion Protein (rIX-FP) in Persons with Hemophilia B: A Single-Center Retrospective\u2013Prospective Study"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3697-706X","authenticated-orcid":false,"given":"Antonio","family":"Coppola","sequence":"first","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianna Franca","family":"Rivolta","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1999-4485","authenticated-orcid":false,"given":"Gabriele","family":"Quintavalle","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5154-4636","authenticated-orcid":false,"given":"Annalisa","family":"Matichecchia","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6308-6054","authenticated-orcid":false,"given":"Federica","family":"Riccardi","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossana","family":"Rossi","sequence":"additional","affiliation":[{"name":"Coagulation Laboratory, Diagnostic Department, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Benegiamo","sequence":"additional","affiliation":[{"name":"Coagulation Laboratory, Diagnostic Department, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Ranalli","sequence":"additional","affiliation":[{"name":"Hemophilia and Blood Rare Disease Center, Oncohematology Department, Spirito Santo Civil Hospital, 65124 Pescara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Coluccio","sequence":"additional","affiliation":[{"name":"Hematology Unit, University Hospital of Modena, 41124 Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annarita","family":"Tagliaferri","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,3,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/hae.14046","article-title":"World Federation of Hemophilia Guidelines for the management of hemophilia","volume":"26","author":"Srivastava","year":"2020","journal-title":"Haemophilia"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1111\/bjh.18379","article-title":"Current and future therapies for haemophilia-Beyond factor replacement therapies","volume":"200","author":"Nogami","year":"2023","journal-title":"Br. 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