{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:20:25Z","timestamp":1789017625802,"version":"build-2803163510"},"reference-count":26,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T00:00:00Z","timestamp":1670284800000},"content-version":"vor","delay-in-days":370,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["rpthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Research and Practice in Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2021,12]]},"DOI":"10.1002\/rth2.12635","type":"journal-article","created":{"date-parts":[[2021,12,21]],"date-time":"2021-12-21T02:17:14Z","timestamp":1640053034000},"page":"e12635","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"title":["2B von Willebrand disease diagnosis: Considerations reflecting on 2021 multisociety guidelines"],"prefix":"10.1016","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7562-203X","authenticated-orcid":false,"given":"Maha","family":"Othman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2103-1661","authenticated-orcid":false,"given":"Emmanuel J.","family":"Favaloro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1","key":"10.1002\/rth2.12635_bb0010","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1182\/bloodadvances.2020003265","article-title":"ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease","volume":"5","author":"James","year":"2021","journal-title":"Blood Adv"},{"key":"10.1002\/rth2.12635_bb0015","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1111\/j.1538-7836.2006.02146.x","article-title":"Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand factor","volume":"4","author":"Sadler","year":"2006","journal-title":"J Thromb Haemost"},{"issue":"4","key":"10.1002\/rth2.12635_bb0020","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1111\/bjh.13064","article-title":"The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology","volume":"167","author":"Laffan","year":"2014","journal-title":"Br J Haematol"},{"issue":"2","key":"10.1002\/rth2.12635_bb0025","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1111\/j.1365-2516.2007.01643.x","article-title":"von Willebrand disease (VWD): evidence\u2010based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)","volume":"14","author":"Nichols","year":"2008","journal-title":"Haemophilia"},{"key":"10.1002\/rth2.12635_bb0030","article-title":"Evaluating errors in the laboratory identification of von Willebrand disease (VWD) using contemporary von Willebrand factor (VWF) assays","author":"Favaloro","year":"2021","journal-title":"Pathology"},{"issue":"5","key":"10.1002\/rth2.12635_bb0035","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1055\/s-0031-1281028","article-title":"Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder","volume":"37","author":"Favaloro","year":"2011","journal-title":"Semin Thromb Hemost"},{"issue":"12","key":"10.1002\/rth2.12635_bb0040","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1182\/blood-2017-06-742692","article-title":"How I treat type 2B von Willebrand disease","volume":"131","author":"Kruse\u2010Jarres","year":"2018","journal-title":"Blood"},{"issue":"1","key":"10.1002\/rth2.12635_bb0045","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1111\/jth.15129","article-title":"Zimmerman Program Investigators. Laboratory variability in the diagnosis of type 2 VWD variants","volume":"19","author":"DiGiandomenico","year":"2021","journal-title":"J Thromb Haemost"},{"issue":"2","key":"10.1002\/rth2.12635_bb0050","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1586\/17474086.2014.868771","article-title":"An update on type 2B von Willebrand disease","volume":"7","author":"Mikhail","year":"2014","journal-title":"Expert Rev Hematol"},{"issue":"4","key":"10.1002\/rth2.12635_bb0055","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.blre.2011.03.003","article-title":"Platelet\u2010type Von Willebrand disease: three decades in the life of a rare bleeding disorder","volume":"25","author":"Othman","year":"2011","journal-title":"Blood Rev"},{"key":"10.1002\/rth2.12635_bb0060","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1007\/978-1-4939-7196-1_35","article-title":"Ristocetin\u2010induced platelet aggregation (RIPA) and RIPA mixing studies","volume":"1646","author":"Frontroth","year":"2017","journal-title":"Methods Mol Biol"},{"issue":"4","key":"10.1002\/rth2.12635_bb0065","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1111\/j.1365-2141.2007.06850.x","article-title":"Differential identification of a rare form of platelet\u2010type (pseudo\u2010) von Willebrand disease (VWD) from Type 2B VWD using a simplified ristocetin\u2010induced\u2010platelet\u2010agglutination mixing assay and confirmed by genetic analysis","volume":"139","author":"Favaloro","year":"2007","journal-title":"Br J Haematol"},{"issue":"1","key":"10.1002\/rth2.12635_bb0070","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1055\/s-2008-1066019","volume":"34","author":"Favaloro","year":"2008","journal-title":"Semin Thromb Hemost"},{"issue":"8","key":"10.1002\/rth2.12635_bb0075","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1111\/jth.14827","article-title":"Guidance on the diagnosis and management of platelet\u2010type von Willebrand disease: a communication from the platelet physiology subcommittee of the ISTH","volume":"18","author":"Othman","year":"2020","journal-title":"J Thromb Haemost"},{"issue":"8","key":"10.1002\/rth2.12635_bb0080","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1177\/1076029614543825","article-title":"Systematic analysis of bleeding phenotype in PT\u2010VWD compared to type 2B VWD using an electronic bleeding questionnaire","volume":"20","author":"Kaur","year":"2014","journal-title":"Clin Appl Thromb Hemost"},{"issue":"3","key":"10.1002\/rth2.12635_bb0085","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1046\/j.1365-2141.2000.02246.x","article-title":"Platelet ultrastructural abnormalities in three patients with type 2B von Willebrand disease","volume":"110","author":"Nurden","year":"2000","journal-title":"Br J Haematol"},{"issue":"9","key":"10.1002\/rth2.12635_bb0090","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1097\/MPH.0b013e31817541cd","article-title":"Type 2B von Willebrand disease associated with the release of platelet agglutinates from megakaryocytes in the bone marrow","volume":"30","author":"Slayton","year":"2008","journal-title":"J Pediatr Hematol Oncol"},{"issue":"12","key":"10.1002\/rth2.12635_bb0095","doi-asserted-by":"crossref","first-page":"5071","DOI":"10.1172\/JCI69458","article-title":"von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin \u03b1IIb\u03b23","volume":"123","author":"Casari","year":"2013","journal-title":"J Clin Invest"},{"issue":"5","key":"10.1002\/rth2.12635_bb0100","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1080\/09537104.2016.1246717","article-title":"Use of a thrombopoietin receptor agonist in von Willebrand disease type 2B (p.V1316M) with severe thrombocytopenia and intracranial hemorrhage","volume":"28","author":"Espitia","year":"2017","journal-title":"Platelets"},{"issue":"5","key":"10.1002\/rth2.12635_bb0105","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1055\/s-0036-1579638","article-title":"Type 2B von Willebrand disease: a matter of plasma plus platelet abnormality","volume":"42","author":"Castaman","year":"2016","journal-title":"Semin Thromb Hemost"},{"issue":"03","key":"10.1002\/rth2.12635_bb0110","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1160\/TH16-04-0317","article-title":"Thrombocytopathy leading to impaired in vivo haemostasis and thrombosis in platelet type von Willebrand disease","volume":"117","author":"Kaur","year":"2017","journal-title":"Thromb Haemost"},{"issue":"7","key":"10.1002\/rth2.12635_bb0115","doi-asserted-by":"crossref","first-page":"1473","DOI":"10.3324\/haematol.2018.200378","article-title":"Mechanisms of thrombocytopenia in platelet\u2010type von Willebrand disease","volume":"104","author":"Bury","year":"2019","journal-title":"Haematologica"},{"issue":"6","key":"10.1002\/rth2.12635_bb0120","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1055\/s-0028-1103363","article-title":"Genetics of type 2B von Willebrand disease: \u201ctrue 2B,\u201d \u201ctricky 2B,\u201d or \u201cnot 2B.\u201d What are the modifiers of the phenotype?","volume":"34","author":"Othman","year":"2008","journal-title":"Semin Thromb Hemost"},{"issue":"5","key":"10.1002\/rth2.12635_bb0125","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1016\/S0025-6196(12)62394-5","article-title":"Von Willebrand factor: structure and function","volume":"66","author":"Meyer","year":"1991","journal-title":"Mayo Clin Proc"},{"issue":"5","key":"10.1002\/rth2.12635_bb0130","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1016\/j.thromres.2015.08.008","article-title":"The co\u2010influence of VWD type 2B\/2M mutations in the A1 domain and platelet GPIb\u03b1 on the rate of cleavage to VWF by ADAMTS13","volume":"136","author":"Ma","year":"2015","journal-title":"Thromb Res"},{"issue":"2","key":"10.1002\/rth2.12635_bb0135","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1111\/j.1538-7836.2007.02296.x","article-title":"Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS\u201013","volume":"5","author":"Rayes","year":"2007","journal-title":"J Thromb Haemost"}],"container-title":["Research and Practice in Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rth2.12635","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/rth2.12635","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037922014911?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037922014911?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rth2.12635","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,11]],"date-time":"2025-11-11T12:22:46Z","timestamp":1762863766000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2475037922014911"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":26,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["S2475037922014911"],"URL":"https:\/\/doi.org\/10.1002\/rth2.12635","relation":{},"ISSN":["2475-0379"],"issn-type":[{"value":"2475-0379","type":"print"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"2B von Willebrand disease diagnosis: Considerations reflecting on 2021 multisociety guidelines","name":"articletitle","label":"Article Title"},{"value":"Research and Practice in Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1002\/rth2.12635","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2021 The Authors. Research and Practice in Thrombosis and Haemostasis published by ELSEVIER INC. on behalf of International Society on Thrombosis and Haemostasis","name":"copyright","label":"Copyright"}],"article-number":"e12635"}}