{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T21:47:35Z","timestamp":1787867255220,"version":"build-2784847793"},"reference-count":21,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,9,12]],"date-time":"2013-09-12T00:00:00Z","timestamp":1378944000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2014,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>von Willebrand disease (VWD) is caused by a quantitative and\/or qualitative deficiency of the von Willebrand factor (VWF). The laboratory diagnosis of VWD is dependent on the measurement of VWF antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo). The aim of this study was to undertake a two\u2010centre evaluation of two new automated VWF:Ag and VWF:RCo assays systems from Instrumentation Laboratory (Bedford, USA). Using the two new analytical systems that operated with different detection principles: immunoturbidimetric (TOP500 analyser) and chemiluminescent (AcuStar analyser), VWF:Ag and VWF:RCo levels were determined in samples from 171 healthy normal subjects, 80 VWD patients (16 type 1, 58 type 2 and 6 type 3) and 7 acquired von Willebrand syndrome patients. With commercial lyophilized normal and pathological plasmas VWF: Ag and VWF:RCo assays performed on both analysers exhibited low levels of inter\u2010assay imprecision (AcuStar: CV% range 3.3\u20136.9; TOP500: CV% range 2.6\u20136.3). Samples from normal healthy subjects (range: VWF:Ag 44.6\u2013173.9\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup>; VWF:RCo 43.1\u2013191.5\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup>) and patients (range: VWF:Ag &lt;0.3\u2013115.1\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup>; VWF:RCo &lt;0.5\u201357.2 IU\u00a0dL<jats:sup>\u22121<\/jats:sup>) showed a good correlation between the two VWF:Ag and VWF:RCo methods (<jats:italic>r<\/jats:italic><jats:sub>s<\/jats:sub>\u00a0=\u00a00.92 and 0.82 respectively), with only a few inconsistent cases among the patients' samples evaluated. The chemiluminescent assays had a lower limit of detection for both VWF:Ag and VWF:RCo compared to immunoturbidimetric tests (0.3\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup> vs. 2.2\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup> and 0.5\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup> vs. 4.4\u00a0IU\u00a0dL<jats:sup>\u22121<\/jats:sup> respectively). The TOP500 and AcuStar VWF:Ag and VWF:RCo assays were precise and compare well between centres, making these systems suitable for the diagnosis of VWD in non\u2010specialized and reference laboratories.<\/jats:p>","DOI":"10.1111\/hae.12264","type":"journal-article","created":{"date-parts":[[2013,9,13]],"date-time":"2013-09-13T04:06:05Z","timestamp":1379045165000},"page":"147-153","source":"Crossref","is-referenced-by-count":42,"title":["A two\u2010centre comparative evaluation of new automated assays for von Willebrand factor ristocetin cofactor activity and antigen"],"prefix":"10.1111","volume":"20","author":[{"given":"F.","family":"Stufano","sequence":"first","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. S.","family":"Lawrie","sequence":"additional","affiliation":[{"name":"Haemostasis Research Unit Department of Haematology University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"La Marca","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Berbenni","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Baronciani","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Peyvandi","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"},{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Universit\u00e0 degli Studi di Milano Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,9,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613992"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1054\/blre.1999.0116"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(09)70150-1"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040403"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.67.1.395"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1538-7836.2003.00260.x"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107465"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.4065\/77.2.181"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_7_11_1","first-page":"38","article-title":"Assay of ristocetin cofactor: experience with a platelet counting technique","volume":"41","author":"Bird AR","year":"1984","journal-title":"Med Lab Sci"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(88)90383-0"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-08-299016"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12064"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.12087"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1651343"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(99)00102-4"},{"key":"e_1_2_7_18_1","first-page":"77","article-title":"A sensitive ristocetin co\u2010factor activity assay with recombinant glycoprotein Ibalpha for the diagnosis of patients with low von Willebrand factor levels","volume":"89","author":"Federici AB","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001721-200408000-00011"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.2307\/2987937"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)91748-9"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-3148.2001.00315.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12264","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12264","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T17:27:51Z","timestamp":1695490071000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12264"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,9,12]]},"references-count":21,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,1]]}},"alternative-id":["10.1111\/hae.12264"],"URL":"https:\/\/doi.org\/10.1111\/hae.12264","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,9,12]]}}}