{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T23:52:08Z","timestamp":1791417128159,"version":"4.3.3"},"reference-count":41,"publisher":"Georg Thieme Verlag KG","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hamostaseologie"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>In recent years, the therapeutic landscape for hemophilia has changed significantly, with the approval of several innovative agents, some featuring entirely novel mechanisms of action. These advances have substantially improved outcomes and reduced the treatment burden. However, clinical experience with these novel therapies in special situations\u2014such as bleeding, surgery, trauma, and infection\u2014remains limited. Factor replacement therapy has been the standard of care for a long time, with extensive clinical experience. The introduction of non-factor therapies has raised important questions: Is additional treatment required after injury or during surgery? What are the considerations in cases of sepsis or disseminated intravascular coagulation (DIC)? This summary focuses on emicizumab, efanesoctocog alfa, newly approved TFPI inhibitors, nonacog beta pegol, and the extension of indications for PEGylated FVIII preparations to younger age groups. We will therefore also briefly address these developments. The largest part of this manuscript will focus on emicizumab due to its longer approval history and the most extensive data available. Most other medications were licensed in 2024 or later.<\/jats:p>","DOI":"10.1055\/a-2957-0168","type":"journal-article","created":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T23:38:13Z","timestamp":1791416293000},"source":"Crossref","is-referenced-by-count":0,"title":["Hemostatic Considerations of New Treatment Options for Hemophilia During Surgery, Infection, and Bleeding in Children and Adolescents"],"prefix":"10.1055","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2421-8947","authenticated-orcid":false,"given":"Ivonne","family":"Wieland","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Lower Saxony, Germany","place":["Hannover"],"department":["Pediatric Hematology and Oncology"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Becker","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Lower Saxony, Germany","place":["Hannover"],"department":["Pediatric Hematology and Oncology"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,10,7]]},"reference":[{"issue":"04","key":"ref2","doi-asserted-by":"publisher","first-page":"1089","DOI":"10.1182\/bloodadvances.2021006119","article-title":"Efanesoctocog alfa for hemophilia a: results from a phase 1 repeat-dose study","volume":"6","author":"T Lissitchkov","year":"2022","journal-title":"Blood Adv"},{"issue":"17","key":"ref3","doi-asserted-by":"publisher","first-page":"1484","DOI":"10.1182\/blood.2019001292","article-title":"BIVV001, a new class of factor VIII replacement for hemophilia a that is independent of von willebrand factor in primates and mice","volume":"135","author":"E Seth Chhabra","year":"2020","journal-title":"Blood"},{"issue":"03","key":"ref4","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1056\/NEJMoa2312611","article-title":"Efanesoctocog alfa prophylaxis for children with severe hemophilia a","volume":"391","author":"L Malec","year":"2024","journal-title":"N Engl J Med"},{"issue":"11","key":"ref5","doi-asserted-by":"publisher","first-page":"3481","DOI":"10.1016\/j.jtha.2025.07.011","article-title":"Post hoc analysis of bleeding episodes and clinically relevant pharmacokinetic parameters among children","volume":"23","author":"L Malec","year":"2025","journal-title":"J Thromb Haemost"},{"issue":"03","key":"ref6","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1111\/hae.70017","article-title":"Perioperative management with efanesoctocog alfa in patients with haemophilia a in the phase 3 XTEND-1 and XTEND-kids studies","volume":"31","author":"R Klamroth","year":"2025","journal-title":"Haemophilia"},{"issue":"01","key":"ref7","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1111\/hae.14831","article-title":"A global comparative field study to evaluate the factor VIII activity of efanesoctocog alfa by one-stage clotting and chromogenic substrate assays at clinical haemostasis laboratories","volume":"30","author":"S Pipe","year":"2024","journal-title":"Haemophilia"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1055\/a-2717-3413","article-title":"On factor VIII assay discrepancies in post-infusion samples obtained from patients treated with efanesoctocog alfa","author":"J M\u00fcller","year":"2026","journal-title":"Hamostaseologie"},{"issue":"03","key":"ref10","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1111\/hae.14294","article-title":"PROTECT VIII kids extension study: long-term safety and efficacy of BAY 94-9027 (damoctocog alfa pegol) in children with severe haemophilia a","volume":"27","author":"M E Mancuso","year":"2021","journal-title":"Haemophilia"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.70059","article-title":"Safety and efficacy of damoctocog alfa pegol in previously treated children aged 7 to","author":"M C Ozelo","year":"2026","journal-title":"Eur J Haematol"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1111\/jth.15036","article-title":"Long-term safety and efficacy of N8-GP in previously treated pediatric patients with hemophilia a: final results from pathfinder5","volume":"18","author":"S \u0160aulyt\u0117 Trakymien\u0117","year":"2020","journal-title":"J Thromb Haemost"},{"issue":"11","key":"ref14","doi-asserted-by":"publisher","first-page":"3109","DOI":"10.1016\/j.jtha.2023.07.030","article-title":"The immunogenicity, safety, and efficacy of N8-GP in previously untreated patients with severe hemophilia a: Pathfinder6 end-of-trial results","volume":"21","author":"G Kenet","year":"2023","journal-title":"J Thromb Haemost"},{"issue":"05","key":"ref16","doi-asserted-by":"publisher","first-page":"737","DOI":"10.1055\/s-0040-1709521","article-title":"Long-term safety and efficacy of nonacog beta pegol (N9-GP) administered for at least 5 years in previously treated children with hemophilia B","volume":"120","author":"M Carcao","year":"2020","journal-title":"Thromb Haemost"},{"issue":"07","key":"ref17","doi-asserted-by":"publisher","first-page":"1101","DOI":"10.1002\/rth2.12412","article-title":"Nonacog beta pegol (N9-GP) in hemophilia B: first report on safety and efficacy in previously untreated and minimally treated patients","volume":"4","author":"A K Chan","year":"2020","journal-title":"Res Pract Thromb Haemost"},{"issue":"02","key":"ref18","doi-asserted-by":"publisher","first-page":"102341","DOI":"10.1016\/j.rpth.2024.102341","article-title":"Nonacog beta pegol prophylaxis in children with hemophilia B: safety, efficacy, and neurodevelopmental outcomes for up to 8 years","volume":"8","author":"K S Walsh","year":"2024","journal-title":"Res Pract Thromb Haemost"},{"issue":"07","key":"ref19","doi-asserted-by":"publisher","first-page":"102208","DOI":"10.1016\/j.rpth.2023.102208","article-title":"Use of nonacog beta pegol during surgery in persons with hemophilia B: a case series","volume":"7","author":"C W Phua","year":"2023","journal-title":"Res Pract Thromb Haemost"},{"issue":"07","key":"ref20","doi-asserted-by":"publisher","first-page":"1348","DOI":"10.1160\/TH17-01-0030","article-title":"Factor VIIIa-mimetic cofactor activity of a bispecific antibody to factors IX\/IXa and X\/xa, emicizumab, depends on its ability to bridge the antigens","volume":"117","author":"T Kitazawa","year":"2017","journal-title":"Thromb Haemost"},{"issue":"06","key":"ref21","doi-asserted-by":"publisher","first-page":"740","DOI":"10.1182\/blood.2019004334","article-title":"A hemophilia a mouse model for the in vivo assessment of emicizumab function","volume":"136","author":"S Ferri\u00e8re","year":"2020","journal-title":"Blood"},{"issue":"24","key":"ref23","doi-asserted-by":"publisher","first-page":"2127","DOI":"10.1182\/blood.2019001869","article-title":"A multicenter, open-label phase 3 study of emicizumab prophylaxis in children with hemophilia a with inhibitors","volume":"134","author":"G Young","year":"2019","journal-title":"Blood"},{"issue":"04","key":"ref24","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1111\/hae.14005","article-title":"Real-world use of emicizumab in patients with haemophilia a: bleeding outcomes and surgical procedures","volume":"26","author":"I McCary","year":"2020","journal-title":"Haemophilia"},{"issue":"04","key":"ref25","doi-asserted-by":"publisher","first-page":"102415","DOI":"10.1016\/j.rpth.2024.102415","article-title":"Emicizumab is well tolerated and effective in people with congenital hemophilia a regardless of age, severity of disease, or inhibitor status: a scoping review","volume":"8","author":"G Young","year":"2024","journal-title":"Res Pract Thromb Haemost"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1111\/hae.70189","article-title":"Outcomes of emicizumab treatment for haemophilia a paediatric patients: a systematic review with meta-analysis","author":"K Bolou","year":"2025","journal-title":"Haemophilia"},{"issue":"08","key":"ref28","doi-asserted-by":"publisher","first-page":"103228","DOI":"10.1016\/j.rpth.2025.103228","article-title":"AOZORA study: 3-year interim analysis of safety and joint health in pediatric people with hemophilia a receiving emicizumab prophylaxis","volume":"9","author":"M Shima","year":"2025","journal-title":"Res Pract Thromb Haemost"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtha.2025.10.029","article-title":"Pharmacokinetic-guided dose reduction of emicizumab in congenital hemophilia a: interim analysis of the DosEmi study","author":"K van der Zwet","year":"2025","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref30","doi-asserted-by":"publisher","first-page":"1375","DOI":"10.1111\/bjh.19312","article-title":"Emicizumab prophylaxis in infants: single-Centre experience","volume":"204","author":"S Levy-Mendelovich","year":"2024","journal-title":"Br J Haematol"},{"issue":"03","key":"ref31","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1111\/hae.14318","article-title":"Emicizumab prophylaxis: prospective longitudinal real-world follow-up and monitoring","volume":"27","author":"A A Barg","year":"2021","journal-title":"Haemophilia"},{"issue":"14","key":"ref32","doi-asserted-by":"publisher","first-page":"1355","DOI":"10.1182\/blood.2023021832","article-title":"Emicizumab prophylaxis in infants with hemophilia a (HAVEN 7): primary analysis of a phase 3b open-label trial","volume":"143","author":"S W Pipe","year":"2024","journal-title":"Blood"},{"issue":"01","key":"ref33","doi-asserted-by":"publisher","first-page":"e32118","DOI":"10.1002\/pbc.32118","article-title":"Emicizumab in previously untreated patients and minimally treated patients with hemophilia a: a comparative study between two international cohorts","volume":"73","author":"S Levy-Mendelovich","year":"2026","journal-title":"Pediatr Blood Cancer"},{"issue":"10","key":"ref34","doi-asserted-by":"publisher","first-page":"E285","DOI":"10.1002\/ajh.27039","article-title":"Severe muscle bleeds in children and young adults with hemophilia a on emicizumab prophylaxis: real-world retrospective multi-institutional cohort","volume":"98","author":"G Batsuli","year":"2023","journal-title":"Am J Hematol"},{"issue":"05","key":"ref35","doi-asserted-by":"publisher","first-page":"e28942","DOI":"10.1002\/pbc.28942","article-title":"Real-world case series and summary of current literature of infants and toddlers with severe hemophilia a with inhibitor on prophylaxis with emicizumab","volume":"68","author":"J Garcia","year":"2021","journal-title":"Pediatr Blood Cancer"},{"issue":"04","key":"ref36","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1007\/s12185-022-03490-x","article-title":"Two pediatric cases of severe hemophilia a in which emicizumab prophylaxis failed to prevent traumatic extra-articular hemorrhage","volume":"117","author":"Y Yamada","year":"2023","journal-title":"Int J Hematol"},{"key":"ref37","doi-asserted-by":"publisher","first-page":"a27818278","DOI":"10.1055\/a-2781-8278","article-title":"Paediatric subanalysis of TSUBASA, assessing physical activity, bleeding, quality of life and safety in people with haemophilia a receiving emicizumab","volume":"10","author":"K Nogami","year":"2026","journal-title":"TH Open"},{"issue":"04","key":"ref38","doi-asserted-by":"publisher","first-page":"e105","DOI":"10.1111\/hae.14574","article-title":"A phase IV, multicentre, open-label study of emicizumab prophylaxis in people with haemophilia a with or without FVIII inhibitors undergoing minor surgical procedures","volume":"28","author":"M Escobar","year":"2022","journal-title":"Haemophilia"},{"key":"ref39","doi-asserted-by":"publisher","first-page":"1155853","DOI":"10.3389\/fped.2023.1155853","article-title":"Management of children with hemophilia a on emicizumab who need surgery","volume":"11","author":"M Belletrutti","year":"2023","journal-title":"Front Pediatr"},{"issue":"09","key":"ref40","doi-asserted-by":"publisher","first-page":"1384","DOI":"10.1055\/s-0039-1692427","article-title":"Laboratory monitoring in emicizumab-treated persons with hemophilia a","volume":"119","author":"J M\u00fcller","year":"2019","journal-title":"Thromb Haemost"},{"issue":"07","key":"ref41","doi-asserted-by":"publisher","first-page":"1084","DOI":"10.1055\/s-0039-1688687","article-title":"Effects and interferences of emicizumab, a humanised bispecific antibody mimicking activated factor VIII cofactor function, on coagulation assays","volume":"119","author":"J I Adamkewicz","year":"2019","journal-title":"Thromb Haemost"},{"issue":"01","key":"ref42","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1111\/hae.14191","article-title":"Management of COVID-19-associated coagulopathy in persons with haemophilia","volume":"27","author":"S W Pipe","year":"2021","journal-title":"Haemophilia"},{"issue":"09","key":"ref43","doi-asserted-by":"publisher","first-page":"881","DOI":"10.1007\/s40265-018-0922-6","article-title":"Inhibition of tissue factor pathway inhibitor (TFPI) as a treatment for haemophilia: rationale with focus on concizumab","volume":"78","author":"P Chowdary","year":"2018","journal-title":"Drugs"},{"issue":"09","key":"ref44","doi-asserted-by":"publisher","first-page":"783","DOI":"10.1056\/NEJMoa2216455","article-title":"Phase 3 trial of concizumab in hemophilia with inhibitors","volume":"389","author":"T Matsushita","year":"2023","journal-title":"N Engl J Med"},{"issue":"12","key":"ref46","doi-asserted-by":"publisher","first-page":"e891","DOI":"10.1016\/S2352-3026(24)00307-7","article-title":"Concizumab prophylaxis in people with haemophilia a or haemophilia B without inhibitors (explorer8): a prospective, multicentre, open-label, randomised, phase 3a trial","volume":"11","author":"P Chowdary","year":"2024","journal-title":"Lancet Haematol"},{"issue":"14","key":"ref48","doi-asserted-by":"publisher","first-page":"1654","DOI":"10.1182\/blood.2024027468","article-title":"Marstacimab prophylaxis in hemophilia a\/B without inhibitors: results from the phase 3 BASIS trial","volume":"146","author":"D Matino","year":"2025","journal-title":"Blood"},{"key":"ref49","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2025031065","article-title":"Efficacy and safety of marstacimab prophylaxis in hemophilia a\/B with inhibitors: results from the phase 3 BASIS trial","author":"D Matino","year":"2025","journal-title":"Blood"}],"container-title":["H\u00e4mostaseologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2957-0168.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T23:38:13Z","timestamp":1791416293000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2957-0168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,7]]},"references-count":41,"URL":"https:\/\/doi.org\/10.1055\/a-2957-0168","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0720-9355","2567-5761"],"issn-type":[{"value":"0720-9355","type":"print"},{"value":"2567-5761","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,7]]}}}