{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T16:45:35Z","timestamp":1773161135818,"version":"3.50.1"},"reference-count":55,"publisher":"Georg Thieme Verlag KG","issue":"03","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Thromb Hemost"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Acquired Hemophilia A (AHA) is a rare bleeding disorder caused by factor VIII inhibitors. Standard therapies are limited by thrombotic risk and prolonged hospitalization. Emicizumab, approved for congenital Hemophilia A, has emerged as a potential alternative in AHA based on case reports and early clinical trial data. To evaluate the efficacy and safety of Emicizumab in AHA through a retrospective real-world case series and a systematic literature review with patient-level data analysis. We retrospectively analyzed five AHA cases treated with Emicizumab at two Italian centers and performed a PRISMA-compliant systematic review of published reports, extracting and analyzing patient-level data using Joanna Briggs Institute tools. In the real-world cohort, early Emicizumab use in five patients with high-titer inhibitors and severe bleeding led to rapid hemorrhagic control, early withdrawal of bypassing agents, and no thrombotic or adverse events. All five patients received immunosuppression, and inhibitor eradication was achieved in 60% of patients, but for 40% follow up is still ongoing. The literature review identified 24 patients from 18 publications. Early Emicizumab administration (at admission) was associated with reduced bleeding recurrence (0% vs. 56.3%), shorter in-hospital stay (median 23.5 days vs. 39 days), and lower bleeding-related mortality (0% vs. 12.5%) compared with delayed administration. Early Emicizumab initiation appears to be a safe and effective strategy for AHA management, particularly in fragile or high-risk populations. Its subcutaneous route, favorable safety profile, and ability to reduce hospitalization support its integration into first-line therapeutic algorithms. Further prospective studies are warranted to define.<\/jats:p>","DOI":"10.1055\/a-2788-1642","type":"journal-article","created":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T00:35:08Z","timestamp":1768437308000},"page":"349-358","source":"Crossref","is-referenced-by-count":0,"title":["Emicizumab in Acquired Hemophilia A: A Real-World Case Series with Patient-Level Outcome Analysis"],"prefix":"10.1055","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8077-2809","authenticated-orcid":false,"given":"Ilenia","family":"Calcaterra","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmine","family":"De Luca","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guido","family":"D'Errico","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ciro","family":"Miele","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Caputo","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raffaele","family":"Russo","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Conca","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ernesto","family":"Cimino","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Guida","sequence":"additional","affiliation":[{"name":"Centre for Congenital Haemorrhagic Disorders and Thrombotic Diseases, Rheumatologic and Immunologic Clinic, San Giovanni di Dio and Ruggi D'Aragona University Hospital, Salerno, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonella","family":"Tufano","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matteo Di","family":"Minno","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2026,1,28]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"691","DOI":"10.2147\/JBM.S342077","article-title":"Acquired haemophilia A: a review of what we know","volume":"13","author":"M E Mingot-Castellano","year":"2022","journal-title":"J Blood Med"},{"issue":"04","key":"ref2","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1111\/j.1538-7836.2012.04654.x","article-title":"Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)","volume":"10","author":"P Knoebl","year":"2012","journal-title":"J Thromb Haemost"},{"issue":"01","key":"ref3","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1007\/s11357-024-01317-7","article-title":"Acquired hemophilia A as a disease of the elderly: a comprehensive review of epidemiology, pathogenesis, and novel therapy","volume":"47","author":"A Lehoczki","year":"2025","journal-title":"Geroscience"},{"issue":"02","key":"ref4","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1111\/hae.14233","article-title":"Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: a systematic literature review","volume":"27","author":"N Dewarrat","year":"2021","journal-title":"Haemophilia"},{"issue":"04","key":"ref5","first-page":"264","article-title":"Role of the hemostasis and thrombosis unit in the management of patients with acquired hemophilia A","volume":"41","author":"A Mameli","year":"2024","journal-title":"Turk J Haematol"},{"issue":"10","key":"ref6","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.3390\/medicina59101739","article-title":"Acquired hemophilia A: bleeding pattern and hemostatic therapeutic strategies","volume":"59","author":"R Marino","year":"2023","journal-title":"Medicina (Kaunas)"},{"issue":"01","key":"ref7","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1182\/hematology.2023000462","article-title":"The role of emicizumab in acquired hemophilia A","volume":"2023","author":"J Poston","year":"2023","journal-title":"Hematology (Am Soc Hematol Educ Program)"},{"issue":"04","key":"ref8","doi-asserted-by":"crossref","first-page":"566","DOI":"10.3324\/haematol.2008.001743","article-title":"International recommendations on the diagnosis and treatment of patients with acquired hemophilia A","volume":"94","author":"A Huth-K\u00fchne","year":"2009","journal-title":"Haematologica"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1186\/1756-0500-3-161","article-title":"Consensus recommendations for the diagnosis and treatment of acquired hemophilia A","volume":"3","author":"P Collins","year":"2010","journal-title":"BMC Res Notes"},{"issue":"07","key":"ref10","doi-asserted-by":"crossref","first-page":"1791","DOI":"10.3324\/haematol.2019.230771","article-title":"International recommendations on the diagnosis and treatment of acquired hemophilia A","volume":"105","author":"A Tiede","year":"2020","journal-title":"Haematologica"},{"issue":"06","key":"ref11","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1111\/bjh.12463","article-title":"Diagnosis and management of acquired coagulation inhibitors: a guideline from UKHCDO","volume":"162","author":"P WCollins","year":"2013","journal-title":"Br J Haematol"},{"issue":"03","key":"ref12","first-page":"245","article-title":"Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding","volume":"20","author":"A Coppola","year":"2022","journal-title":"Blood Transfus"},{"issue":"03","key":"ref13","doi-asserted-by":"crossref","first-page":"420","DOI":"10.3390\/diagnostics13030420","article-title":"Acquired hemophilia A: an update on the etiopathogenesis, diagnosis, and treatment","volume":"13","author":"E Zanon","year":"2023","journal-title":"Diagnostics (Basel)"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"100310","DOI":"10.1016\/j.bjao.2024.100310","article-title":"Perioperative considerations in the paediatric patient with congenital and acquired coagulopathy","volume":"12","author":"G Erdoes","year":"2024","journal-title":"BJA Open"},{"issue":"07","key":"ref15","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1182\/blood-2014-07-587089","article-title":"Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01\/2010 study","volume":"125","author":"A Tiede","year":"2015","journal-title":"Blood"},{"issue":"05","key":"ref16","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1055\/a-2384-3585","article-title":"Final analysis results from the AGEHA study: emicizumab prophylaxis for acquired hemophilia A with or without immunosuppressive therapy","volume":"125","author":"M Shima","year":"2025","journal-title":"Thromb Haemost"},{"issue":"04","key":"ref17","doi-asserted-by":"crossref","first-page":"102438","DOI":"10.1016\/j.rpth.2024.102438","article-title":"Emicizumab as first-line therapy in acquired hemophilia A","volume":"8","author":"M Iarossi","year":"2024","journal-title":"Res Pract Thromb Haemost"},{"issue":"01","key":"ref18","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1111\/hae.14684","article-title":"Safety of FEIBA and emicizumab (SAFE): Dose escalation study evaluating the safety of in vivo administration of activated prothrombin complex concentrate in haemophilia A patients on emicizumab","volume":"29","author":"H Kizilocak","year":"2023","journal-title":"Haemophilia"},{"issue":"03","key":"ref19","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1016\/j.jtha.2022.10.004","article-title":"A prospective, multicenter, open-label phase III study of emicizumab prophylaxis in patients with acquired hemophilia A","volume":"21","author":"M Shima","year":"2023","journal-title":"J Thromb Haemost"},{"issue":"11","key":"ref20","doi-asserted-by":"crossref","first-page":"e913","DOI":"10.1016\/S2352-3026(23)00280-6","article-title":"Emicizumab prophylaxis in patients with acquired haemophilia A (GTH-AHA-EMI): an open-label, single-arm, multicentre, phase 2 study","volume":"10","author":"A Tiede","year":"2023","journal-title":"Lancet Haematol"},{"issue":"10","key":"ref21","doi-asserted-by":"crossref","first-page":"2692","DOI":"10.1016\/j.jtha.2024.06.010","article-title":"Emicizumab versus immunosuppressive therapy for the management of acquired hemophilia A","volume":"22","author":"C Hart","year":"2024","journal-title":"J Thromb Haemost"},{"issue":"22","key":"ref22","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1182\/bloodadvances.2017006684","article-title":"Long-term safety and efficacy of emicizumab in a phase 1\/2 study in patients with hemophilia A with or without inhibitors","volume":"1","author":"M Shima","year":"2017","journal-title":"Blood Adv"},{"issue":"03","key":"ref23","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1111\/hae.13990","article-title":"Effects of emicizumab on APTT, one-stage and chromogenic assays of factor VIII in artificially spiked plasma and in samples from haemophilia A patients with inhibitors","volume":"26","author":"A Bowyer","year":"2020","journal-title":"Haemophilia"},{"issue":"71","key":"ref24","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: an updated guideline for reporting systematic reviews","volume":"372","author":"M J Page","year":"2021","journal-title":"BMJ"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1177\/20406207211038193","article-title":"Emicizumab in acquired haemophilia A: about two clinical cases and literature review","volume":"12","author":"A Hansenne","year":"2021","journal-title":"Ther Adv Hematol"},{"issue":"06","key":"ref27","doi-asserted-by":"crossref","first-page":"103457","DOI":"10.1016\/j.transci.2022.103457","article-title":"Utilization of emicizumab in acquired hemophilia A: a case report","volume":"61","author":"S G Yates","year":"2022","journal-title":"Transfus Apher Sci"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.12659\/AJCR.916783","article-title":"Emicizumab use in treatment of acquired hemophilia A: a case report","volume":"20","author":"K Al-Banaa","year":"2019","journal-title":"Am J Case Rep"},{"issue":"02","key":"ref29","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1002\/jha2.878","article-title":"Emicizumab for acquired hemophilia A: report of two cases and dosing strategies","volume":"5","author":"F Ahmed","year":"2024","journal-title":"eJHaem"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ijscr.2021.01.038","article-title":"Unforeseen encounter of acquired hemophilia A in a preoperative case of periampullary carcinoma: a case report","volume":"79","author":"S S Jena","year":"2021","journal-title":"Int J Surg Case Rep"},{"issue":"01","key":"ref31","doi-asserted-by":"crossref","first-page":"T45","DOI":"10.1016\/j.farma.2023.10.002","article-title":"[Translated article] Utilization of emicizumab in the treatment of a case of acquired haemophilia A","volume":"48","author":"M A Oca\u00f1a G\u00f3mez","year":"2024","journal-title":"Farm Hosp"},{"issue":"08","key":"ref32","doi-asserted-by":"crossref","first-page":"e31083","DOI":"10.1002\/pbc.31083","article-title":"Successful management of an adolescent male with acquired hemophilia-A with intracranial bleeding using emicizumab","volume":"71","author":"N Ragavan","year":"2024","journal-title":"Pediatr Blood Cancer"},{"issue":"04","key":"ref33","doi-asserted-by":"crossref","first-page":"e502","DOI":"10.1111\/hae.14225","article-title":"Persistent inhibitor in acquired haemophilia A: a case for emicizumab?","volume":"27","author":"M Ganslmeier","year":"2021","journal-title":"Haemophilia"},{"issue":"02","key":"ref34","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1159\/000497287","article-title":"Emicizumab in the treatment of acquired haemophilia: a case report","volume":"46","author":"P M\u00f6hnle","year":"2019","journal-title":"Transfus Med Hemother"},{"issue":"06","key":"ref35","doi-asserted-by":"crossref","first-page":"e242876","DOI":"10.1136\/bcr-2021-242876","article-title":"Acquired haemophilia A: successful treatment of a patient using upfront immunosuppressive therapy and haemostatic agents","volume":"14","author":"S Y Chung","year":"2021","journal-title":"BMJ Case Rep"},{"issue":"03","key":"ref36","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1111\/jth.15208","article-title":"Should emicizumab be used in patients with acquired hemophilia A?","volume":"19","author":"A Tiede","year":"2021","journal-title":"J Thromb Haemost"},{"issue":"02","key":"ref37","doi-asserted-by":"crossref","first-page":"e12688","DOI":"10.1002\/rth2.12688","article-title":"Recombinant porcine factor VIII in acquired hemophilia A: experience from two patients and literature review","volume":"6","author":"A Hayden","year":"2022","journal-title":"Res Pract Thromb Haemost"},{"issue":"11","key":"ref38","first-page":"002984","article-title":"Anticoagulation for stroke prevention after restoration of haemostasis with emicizumab in acquired haemophilia A","volume":"8","author":"K Al-Banaa","year":"2021","journal-title":"Eur J Case Rep Intern Med"},{"issue":"03","key":"ref39","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1002\/rth2.12201","article-title":"Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention","volume":"3","author":"K E Dane","year":"2019","journal-title":"Res Pract Thromb Haemost"},{"issue":"02","key":"ref40","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1111\/bcp.15598","article-title":"Management of acquired haemophilia A in severe COVID-19: haemostatic bridging with emicizumab to keep the balance between bleeding and thrombosis","volume":"89","author":"G Gelbenegger","year":"2023","journal-title":"Br J Clin Pharmacol"},{"issue":"03","key":"ref41","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.jtha.2023.01.010","article-title":"Hemorrhagic and thrombotic adverse events associated with emicizumab and extended half-life factor VIII replacement drugs: EudraVigilance data of 2021","volume":"21","author":"M Abbattista","year":"2023","journal-title":"J Thromb Haemost"},{"key":"ref42","first-page":"e922326","article-title":"Utilization of emicizumab in acquired factor VIII deficiency","volume":"21","author":"K J Hess","year":"2020","journal-title":"Am J Case Rep"},{"issue":"06","key":"ref43","doi-asserted-by":"crossref","first-page":"e12785","DOI":"10.1002\/rth2.12785","article-title":"Acquired hemophilia following COVID-19 vaccination: case report and review of literature","volume":"6","author":"M Happaerts","year":"2022","journal-title":"Res Pract Thromb Haemost"},{"issue":"06","key":"ref44","first-page":"549","article-title":"Emicizumab in acquired hemophilia A: pros and cons of a new approach to the prevention and treatment of bleeding","volume":"21","author":"S Pasca","year":"2023","journal-title":"Blood Transfus"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1177\/10760296241298661","article-title":"Outcomes of emicizumab in acquired hemophilia patients: a systematic review","volume":"30","author":"G Ikbel","year":"2024","journal-title":"Clin Appl Thromb Hemost"},{"issue":"03","key":"ref46","doi-asserted-by":"crossref","first-page":"302","DOI":"10.3164\/jcbn.20-85","article-title":"Risk for prolonged hospitalization and mortality in aged community acquired pneumonia patients: a retrospective study in Japan","volume":"67","author":"A Matsuo","year":"2020","journal-title":"J Clin Biochem Nutr"},{"issue":"01","key":"ref47","first-page":"e190","article-title":"Cost of care of haemophilia with inhibitors","volume":"16","author":"M N Di Minno","year":"2010","journal-title":"Haemophilia"},{"issue":"04","key":"ref48","doi-asserted-by":"crossref","first-page":"102926","DOI":"10.1016\/j.rpth.2025.102926","article-title":"Activated prothrombin complex concentrate in patients receiving emicizumab prophylaxis: from evidence to clinical practice","volume":"9","author":"R F Sidonio Jr","year":"2025","journal-title":"Res Pract Thromb Haemost"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"109422","DOI":"10.1016\/j.thromres.2025.109422","article-title":"A real-world pharmacovigilance analysis of the FDA adverse event reporting system database for emicizumab","volume":"254","author":"T Li","year":"2025","journal-title":"Thromb Res"},{"issue":"01","key":"ref50","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1186\/s12872-021-02039-w","article-title":"Prognosis and management of new-onset atrial fibrillation in critically ill patients","volume":"21","author":"J Qian","year":"2021","journal-title":"BMC Cardiovasc Disord"},{"issue":"26","key":"ref51","doi-asserted-by":"crossref","first-page":"1726","DOI":"10.1093\/eurheartj\/ehu197","article-title":"Importance of frailty in patients with cardiovascular disease","volume":"35","author":"M Singh","year":"2014","journal-title":"Eur Heart J"},{"issue":"01","key":"ref52","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1186\/s12884-024-06387-1","article-title":"Development and validation of the physical exercise program \u201cactive mums\u201d for postpartum recovery: application of the CReDECI-2 guidelines","volume":"24","author":"C Brites-Lagos","year":"2024","journal-title":"BMC Pregnancy Childbirth"},{"issue":"01","key":"ref53","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.ajic.2015.08.018","article-title":"Patient volumes and pre- and postdischarge postpartum infection: a retrospective cohort study","volume":"44","author":"A M Parriott","year":"2016","journal-title":"Am J Infect Control"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.jpeds.2017.08.069","article-title":"Delayed breastfeeding initiation is associated with infant morbidity","volume":"191","author":"E R Smith","year":"2017","journal-title":"J Pediatr"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1016\/j.jad.2022.04.091","article-title":"Association between breastfeeding and postpartum depression: a meta-analysis","volume":"308","author":"M Xia","year":"2022","journal-title":"J Affect Disord"},{"issue":"01","key":"ref56","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.jtha.2024.10.003","article-title":"Comprehensive evaluation of anti-emicizumab antibodies in acquired hemophilia A: a detailed case study and methodological evaluation","volume":"23","author":"B Pezeshkpoor","year":"2025","journal-title":"J Thromb Haemost"}],"container-title":["Seminars in Thrombosis and Hemostasis"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2788-1642.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T00:04:29Z","timestamp":1773101069000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2788-1642"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,14]]},"references-count":55,"journal-issue":{"issue":"03","published-online":{"date-parts":[[2026,3,9]]},"published-print":{"date-parts":[[2026,4]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2788-1642","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0094-6176","1098-9064"],"issn-type":[{"value":"0094-6176","type":"print"},{"value":"1098-9064","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,1,14]]},"published":{"date-parts":[[2026,1,14]]}}}