{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T05:01:03Z","timestamp":1771304463320,"version":"3.50.1"},"reference-count":34,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,6,21]],"date-time":"2021-06-21T00:00:00Z","timestamp":1624233600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,6,21]],"date-time":"2021-06-21T00:00:00Z","timestamp":1624233600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Sci Rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To prevent bleeding in severe haemophilia A [SHA, defined as factor VIII (FVIII) activity\u2009&lt;\u20091%] regular prophylactic FVIII replacement therapy is required, and the benefits of factor products with extended half-life (EHL) over traditional standard half-life (SHL) are still being debated. We performed a multi-centre, retrospective cohort study of persons with SHA in Austria aiming to compare clinical outcomes and factor utilization in patients with SHA, who switched from prophylaxis with SHL to an EHL. Data were collected from haemophilia-specific patient diaries and medical records. Twenty male persons with SHA (median age: 32.5\u00a0years) were included. The most common reason for switching to the EHL was a high bleeding rate with SHL. Switch to rFVIII-Fc resulted in a significantly decreased annualized bleeding rate (ABR; median difference (IQR): \u2212\u00a00.3 (\u2212\u00a04.5\u20130); Wilcoxon signed-rank test for matched pairs: Z\u2009=\u2009\u2212\u00a02.7, p\u2009=\u20090.008) and number of prophylactic infusions per week (\u2212\u00a00.75 (\u2212\u00a01.0\u20130.0); Z\u2009=\u2009\u2212\u00a02.7, p\u2009=\u20090.007). Factor utilization was comparable to prior prophylaxis with SHL (0.0 (\u2212\u00a015.8\u201324.8) IU\/kg\/week; Z\u2009=\u2009\u2212\u00a00.4, p\u2009=\u20090.691). In summary, switch to EHL (rFVIII-Fc) was associated with an improved clinical outcome, reflected by ABR reduction, and less frequent infusions, without significantly higher factor usage.<\/jats:p>","DOI":"10.1038\/s41598-021-92245-5","type":"journal-article","created":{"date-parts":[[2021,6,21]],"date-time":"2021-06-21T10:03:10Z","timestamp":1624269790000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Bleeding outcomes and factor utilization after switching to an extended half-life product for prophylaxis in haemophilia A in Austria"],"prefix":"10.1038","volume":"11","author":[{"given":"Cihan","family":"Ay","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clemens","family":"Feistritzer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joachim","family":"Rettl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerhard","family":"Schuster","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Vavrovsky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonard","family":"Perschy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ingrid","family":"Pabinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,6,21]]},"reference":[{"key":"92245_CR1","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1016\/S0140-6736(15)01123-X","volume":"388","author":"F Peyvandi","year":"2016","unstructured":"Peyvandi, F., Garagiola, I. & Young, G. The past and future of haemophilia: Diagnosis, treatments, and its complications. Lancet 388, 187\u2013197 (2016).","journal-title":"Lancet"},{"key":"92245_CR2","doi-asserted-by":"publisher","first-page":"e1","DOI":"10.1111\/j.1365-2516.2012.02909.x","volume":"19","author":"A Srivastava","year":"2013","unstructured":"Srivastava, A. et al. Guidelines for the management of hemophilia. Haemophilia 19, e1\u2013e47 (2013).","journal-title":"Haemophilia"},{"key":"92245_CR3","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1007\/s00508-015-0872-x","volume":"127","author":"I Pabinger","year":"2015","unstructured":"Pabinger, I. et al. H\u00e4mophiliebehandlung in \u00d6sterreich. Wien. Klin. Wochenschr. 127, 115\u2013130 (2015).","journal-title":"Wien. Klin. Wochenschr."},{"key":"92245_CR4","doi-asserted-by":"publisher","DOI":"10.4274\/tjh.galenos.2019.2018.0393","author":"MC Ar","year":"2019","unstructured":"Ar, M. C., Balkan, C. & Kavakl\u0131, K. Extended half-life (EHL) coagulation factors: A new era in the management of haemophilia patients. Turk. J. Hematol. https:\/\/doi.org\/10.4274\/tjh.galenos.2019.2018.0393 (2019).","journal-title":"Turk. J. Hematol."},{"key":"92245_CR5","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2019.12.012","author":"P Chowdary","year":"2019","unstructured":"Chowdary, P. Extended half-life recombinant products in haemophilia clinical practice\u2014Expectations, opportunities and challenges. Thromb. Res. https:\/\/doi.org\/10.1016\/j.thromres.2019.12.012 (2019).","journal-title":"Thromb. Res."},{"key":"92245_CR6","doi-asserted-by":"publisher","first-page":"946","DOI":"10.1111\/hae.13834","volume":"25","author":"F Peyvandi","year":"2019","unstructured":"Peyvandi, F. et al. Real-life experience in switching to new extended half-life products at European haemophilia centres. Haemophilia 25, 946\u2013952 (2019).","journal-title":"Haemophilia"},{"key":"92245_CR7","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1159\/000488060","volume":"45","author":"L Graf","year":"2018","unstructured":"Graf, L. Extended half-life factor VIII and factor IX preparations. Transfus. Med. Hemother. 45, 86\u201391 (2018).","journal-title":"Transfus. Med. Hemother."},{"key":"92245_CR8","doi-asserted-by":"publisher","first-page":"018","DOI":"10.1055\/s-0035-1568877","volume":"42","author":"R Kumar","year":"2016","unstructured":"Kumar, R., Dunn, A. & Carcao, M. Changing paradigm of hemophilia management: Extended half-life factor concentrates and gene therapy. Semin. Thromb. Hemost. 42, 018\u2013029 (2016).","journal-title":"Semin. Thromb. Hemost."},{"key":"92245_CR9","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1177\/2040620718796429","volume":"9","author":"T Lambert","year":"2018","unstructured":"Lambert, T. et al. Practical aspects of extended half-life products for the treatment of haemophilia. Ther. Adv. Hematol. 9, 295\u2013308 (2018).","journal-title":"Ther. Adv. Hematol."},{"key":"92245_CR10","doi-asserted-by":"publisher","first-page":"317","DOI":"10.1182\/blood-2013-10-529974","volume":"123","author":"J Mahlangu","year":"2014","unstructured":"Mahlangu, J. et al. Phase 3 study of recombinant factor VIII Fc fusion protein in severe hemophilia A. Blood 123, 317\u2013325 (2014).","journal-title":"Blood"},{"key":"92245_CR11","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1111\/hae.13246","volume":"23","author":"K Hampton","year":"2017","unstructured":"Hampton, K. et al. First report on the safety and efficacy of an extended half-life glycoPEGylated recombinant FVIII for major surgery in severe haemophilia A. Haemophilia 23, 689\u2013696 (2017).","journal-title":"Haemophilia"},{"key":"92245_CR12","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1111\/jth.13597","volume":"15","author":"MT Reding","year":"2017","unstructured":"Reding, M. T. et al. Safety and efficacy of BAY 94\u20139027, a prolonged-half-life factor VIII. J. Thromb. Haemost. 15, 411\u2013419 (2017).","journal-title":"J. Thromb. Haemost."},{"key":"92245_CR13","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1111\/hae.13712","volume":"25","author":"N Curry","year":"2019","unstructured":"Curry, N. et al. Once-weekly prophylaxis with glycoPEGylated recombinant factor VIII (N8-GP) in severe haemophilia A: Safety and efficacy results from pathfinder 2 (randomized phase III trial). Haemophilia 25, 373\u2013381 (2019).","journal-title":"Haemophilia"},{"issue":"10","key":"92245_CR14","first-page":"39","volume":"27","author":"A Chhabra","year":"2018","unstructured":"Chhabra, A. et al. 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Expert Rev. Pharmacoecon. Outcomes Res. https:\/\/doi.org\/10.1080\/14737167.2020.1789457 (2020).","journal-title":"Expert Rev. Pharmacoecon. Outcomes Res."},{"key":"92245_CR17","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1111\/hae.13432","volume":"24","author":"C Wang","year":"2018","unstructured":"Wang, C. & Young, G. Clinical use of recombinant factor VIII Fc and recombinant factor IX Fc in patients with haemophilia A and B. Haemophilia 24, 414\u2013419 (2018).","journal-title":"Haemophilia"},{"key":"92245_CR18","doi-asserted-by":"publisher","DOI":"10.2450\/2019.0220-19","author":"A Tagliaferri","year":"2019","unstructured":"Tagliaferri, A. et al. Optimising prophylaxis outcomes and costs in haemophilia patients switching to recombinant FVIII-Fc: A single-centre real-world experience. 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Haemophilia 24, 253\u2013260 (2018).","journal-title":"Haemophilia"}],"container-title":["Scientific Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-92245-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-92245-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-92245-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,3]],"date-time":"2022-12-03T10:07:42Z","timestamp":1670062062000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-92245-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,21]]},"references-count":34,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,12]]}},"alternative-id":["92245"],"URL":"https:\/\/doi.org\/10.1038\/s41598-021-92245-5","relation":{},"ISSN":["2045-2322"],"issn-type":[{"value":"2045-2322","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,21]]},"assertion":[{"value":"22 November 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 June 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 June 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"C.A. received personal fees from Bayer, CSL Behring, Shire\/Takeda, Novo Nordisk, Octapharma, Roche and Sobi for consultancy or\/and participation in speaker bureau or\/and advisory boards. C.F. received personal fees from Bayer, Shire\/Takeda, Novo Nordisk, Roche and Sobi for consultancy or\/and participation in speaker bureau or\/and advisory boards. J.R. received personal fees from Bayer, Shire\/Takeda, Novo Nordisk, Roche, Amgen, CSL Behring and Sobi for consultancy or\/and advisory boards. G.S. received personal fees from Bayer, Novo Nordisk, Roche and Sobi for consultancy or\/and participation in speaker bureau or\/and advisory boards. A.V. is employed at the Academy for Value in Health GmbH and confirms that there are no other relevant financial or non-financial competing interests to report. L.P. received personal fees from Sobi. I.P. received personal fees from Bayer, Biotest, CSL Behring, Shire\/Takeda, Novo Nordisk, Octapharma, Roche and Sobi for lectures or\/and advisory board meetings.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"12967"}}