{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T19:53:27Z","timestamp":1772999607074,"version":"3.50.1"},"reference-count":47,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,2,9]],"date-time":"2025-02-09T00:00:00Z","timestamp":1739059200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,2,9]],"date-time":"2025-02-09T00:00:00Z","timestamp":1739059200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Von Willebrand disease (VWD) is an inherited bleeding disorder caused by deficient or dysfunctional von Willebrand factor (VWF). VWF replacement therapy is indicated in VWD management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This systematic review was conducted to evaluate all available evidence of the efficacy, safety, dosing and consumption of pasteurized plasma\u2010derived human coagulation FVIII\/human VWF (pdVWF\/FVIII; Haemate P\/Humate\u2010P) concentrate for on\u2010demand (OD) treatment, surgical prophylaxis and long\u2010term prophylaxis of patients with VWD. A systematic search was performed in MEDLINE and Cochrane Library databases to identify studies (7 June 1982\u201331 May 2023) reporting the use of pdVWF\/FVIII in VWD according to predefined selection criteria. Pharmacovigilance data were also retrieved for the same period.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Fifteen studies were identified, 12 being observational and three interventional. Efficacy and safety assessments and treatment protocols varied across the studies which hindered direct comparisons. Haemostatic efficacy of pdVWF\/FVIII was rated excellent\/good for OD treatment in 95%\u201398% of bleeds and in 94%\u2013100% of surgeries. In two separate studies, prophylactic efficacy was rated excellent\/good in 100% of treatment cycles. Where reported, median annualized bleeding rates decreased from 3\u201324\u00a0prior prophylaxis to 0.5\u20136 during prophylaxis. Analysis of pharmacovigilance safety reports showed that pdVWF\/FVIII was associated with a low rate of adverse events.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This systematic literature review and analysis of pharmacovigilance data summarize evidence of over 40 years of clinical use of pdVWF\/FVIII, supporting its safety and efficacy in VWD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.15138","type":"journal-article","created":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T06:58:25Z","timestamp":1739257105000},"page":"247-262","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Plasma\u2010Derived von Willebrand Factor\/Factor VIII Concentrate (Haemate P) in von Willebrand Disease: A Systematic Review and Pharmacovigilance Update"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0802-5280","authenticated-orcid":false,"given":"Carmen Escuriola","family":"Ettingshausen","sequence":"first","affiliation":[{"name":"Haemophilia Centre Rhein Main (HZRM) Frankfurt\/Main Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1911-3094","authenticated-orcid":false,"given":"Riitta","family":"Lassila","sequence":"additional","affiliation":[{"name":"Department of Haematology Helsinki University Hospital Coagulation Disorders Unit, Comprehensive Cancer Centre, and Research Program Unit in System Oncology University of Helsinki Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6658-0381","authenticated-orcid":false,"given":"Gines","family":"Escolar","sequence":"additional","affiliation":[{"name":"Department of Pathology Hematopathology Centre de Diagnostic Biomedic (CDB) Hospital Clinic de Barcelona Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS) Universitat de Barcelona Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4989-6421","authenticated-orcid":false,"given":"Christoph","family":"Male","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Medical University of Vienna Vienna Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4705-8475","authenticated-orcid":false,"given":"Kathrin","family":"Schirner","sequence":"additional","affiliation":[{"name":"CSL Innovation GmbH Marburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0977-6422","authenticated-orcid":false,"given":"Lisa","family":"Heyder","sequence":"additional","affiliation":[{"name":"CSL Innovation GmbH Marburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1337-7195","authenticated-orcid":false,"given":"Erik","family":"Berntorp","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine Clinical Coagulation Research Unit Lund University Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,2,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020003265"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03661.x"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2008.01049.x"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.077263"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1601561"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.21037\/aob.2018.03.04"},{"key":"e_1_2_10_8_1","unstructured":"CSLBehring.Humate\u2010P Prescribing Information accessed May 31 2023 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