{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T02:44:41Z","timestamp":1785465881072,"version":"3.56.0"},"reference-count":33,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,4,27]],"date-time":"2022-04-27T00:00:00Z","timestamp":1651017600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","award":["18K07885"],"award-info":[{"award-number":["18K07885"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","award":["26461593"],"award-info":[{"award-number":["26461593"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010795","name":"Chugai Pharmaceutical","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010795","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Type 1 and type 3 von Willebrand disease (VWD) are caused by partial and complete, quantitative deficiency of von Willebrand factor (VWF), respectively, and factor (F)VIII\/VWF complex concentrates are used for haemostatic treatment. Emicizumab, mimics activated FVIII, reduces bleeding in haemophilia A patients. The effects of emicizumab on haemostasis in both types of VWD remain to be fully established, however.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To examine the effects of emicizumab on thrombogenesis in type 1 and type 3 VWD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients\/Methods<\/jats:title><jats:p>Perfusion chamber experiments under high shear conditions (2500\u00a0s<jats:sup>\u20131<\/jats:sup>) combined with immunostaining were performed using whole blood samples from patients with type 1 (VWF:Ag 25 U\/dl) and type 3 VWD (&lt;1.0 U\/dl).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The addition of FVIII (1 U\/ml) to type 1 blood did not affect thrombus formation, whilst supplementation with VWF (1.6 U\/ml) or FVIII\/VWF (1 U\/ml\/1.6 U\/ml) enhanced thrombogenesis to a similar extent. FVIII\/VWF promoted thrombus formation significantly more than VWF alone, however, in type 3 blood. Emicizumab (100 \u03bcg\/ml) augmented thrombus formation in type 3 blood compared to FVIII, and this potency seemed to be somewhat greater than that of VWF. Surface coverage of formed thrombus in type 3 VWD was less than that in type 1 VWD, but thrombus height was comparable in both. The addition of emicizumab to type 3 blood enhanced thrombin generation and fibrin formation compared to control IgG.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Emicizumab promoted mechanisms of thrombus formation in vitro in type 3 and type 1 VWD, suggesting the possibility of alternative therapeutic protocols in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14581","type":"journal-article","created":{"date-parts":[[2022,4,28]],"date-time":"2022-04-28T06:24:16Z","timestamp":1651127056000},"page":"694-701","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Emicizumab enhances thrombus formation in vitro under high shear flow conditions in whole blood from patients with type 1 and type 3 von Willebrand disease"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6003-7276","authenticated-orcid":false,"given":"Hiroaki","family":"Yaoi","sequence":"first","affiliation":[{"name":"Department of Pediatrics Nara Medical University  Kashihara Nara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuaki","family":"Shida","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University  Kashihara Nara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3046-6915","authenticated-orcid":false,"given":"Kenichi","family":"Ogiwara","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University  Kashihara Nara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takehisa","family":"Kitazawa","sequence":"additional","affiliation":[{"name":"Research Division Chugai Pharmaceutical Co.  Gotemba Shizuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5922-7061","authenticated-orcid":false,"given":"Midori","family":"Shima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University  Kashihara Nara Japan"},{"name":"Thrombosis and Hemostasis Research Center Nara Medical University  Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2415-2194","authenticated-orcid":false,"given":"Keiji","family":"Nogami","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University  Kashihara Nara 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