{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T00:17:27Z","timestamp":1793146647875,"version":"4.5.0"},"reference-count":18,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["jthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2026,5]]},"DOI":"10.1016\/j.jtha.2026.01.023","type":"journal-article","created":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T16:48:59Z","timestamp":1771001339000},"page":"1881-1885","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"title":["Caplacizumab resistance in immune thrombotic thrombocytopenic purpura is associated with a von Willebrand factor A1 domain missense variant"],"prefix":"10.1016","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9288-7259","authenticated-orcid":false,"given":"Mari","family":"Thomas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imogen","family":"Buckle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adela","family":"Constantinescu-Bercu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rens","family":"De Groot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"McCann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Scully","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jtha.2026.01.023_bib1","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1056\/NEJMoa1806311","article-title":"Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura","volume":"380","author":"Scully","year":"2019","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jtha.2026.01.023_bib2","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1111\/j.1365-2141.2005.05420.x","article-title":"FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay","volume":"129","author":"Kokame","year":"2005","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jtha.2026.01.023_bib3","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1111\/j.1365-2141.2006.06448.x","article-title":"Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13","volume":"136","author":"Scully","year":"2007","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jtha.2026.01.023_bib4","doi-asserted-by":"crossref","first-page":"3185","DOI":"10.1016\/j.jtha.2025.06.027","article-title":"A microfluidic approach reveals ongoing disease activity in thrombotic thrombocytopenic purpura patients despite clinical remission","volume":"23","author":"Constantinescu-Bercu","year":"2025","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2026.01.023_bib5","doi-asserted-by":"crossref","first-page":"2360","DOI":"10.1038\/s41467-021-22634-x","article-title":"Activation of von Willebrand factor via mechanical unfolding of its discontinuous autoinhibitory module","volume":"12","author":"Arce","year":"2021","journal-title":"Nat Commun"},{"key":"10.1016\/j.jtha.2026.01.023_bib6","doi-asserted-by":"crossref","first-page":"2221","DOI":"10.1073\/pnas.91.6.2221","article-title":"Multiple substitutions in the von Willebrand factor gene that mimic the pseudogene sequence","volume":"91","author":"Eikenboom","year":"1994","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jtha.2026.01.023_bib7","doi-asserted-by":"crossref","first-page":"2636","DOI":"10.1182\/blood-2023-179316","article-title":"Caplacizumab frontline added to therapeutic plasma exchange and immunosuppression prevents unfavorable outcomes in immune-mediated TTP: an international real-world study of the TTP-IWG (The Capla 500 Project)","volume":"142","author":"Coppo","year":"2023","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2026.01.023_bib8","doi-asserted-by":"crossref","first-page":"2169","DOI":"10.1172\/JCI116443","article-title":"von Willebrand factor mutation enhancing interaction with platelets in patients with normal multimeric structure","volume":"91","author":"Holmberg","year":"1993","journal-title":"J Clin Invest"},{"key":"10.1016\/j.jtha.2026.01.023_bib9","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1111\/j.1538-7836.2007.02349.x","article-title":"Biochemical characterization of a recombinant von Willebrand factor (VWF) with combined type 2B and type 1 defects in the VWF gene in two patients with a type 2A phenotype of von Willebrand disease","volume":"5","author":"Baronciani","year":"2007","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2026.01.023_bib10","doi-asserted-by":"crossref","first-page":"610","DOI":"10.3324\/haematol.2009.005751","article-title":"Clinical and laboratory versus molecular markers for a correct classification of von Willebrand disease","volume":"94","author":"Federici","year":"2009","journal-title":"Haematologica"},{"key":"10.1016\/j.jtha.2026.01.023_bib11","first-page":"92","article-title":"Phenotypic parameters in genotypically selected type 2B von Willebrand disease patients: a large, single-center experience including a new novel mutation","volume":"43","author":"Woods","year":"2017","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.jtha.2026.01.023_bib12","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0179566","article-title":"Type 2B von Willebrand disease with or without large multimers: a distinction of the two sides of the disorder is long overdue","volume":"12","author":"Casonato","year":"2017","journal-title":"PLoS One"},{"key":"10.1016\/j.jtha.2026.01.023_bib13","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1038\/s41586-023-06045-0","article-title":"A genomic mutational constraint map using variation in 76,156 human genomes","volume":"625","author":"Chen","year":"2024","journal-title":"Nature"},{"key":"10.1016\/j.jtha.2026.01.023_bib14","doi-asserted-by":"crossref","first-page":"17847","DOI":"10.1002\/jcb.29052","article-title":"Effects of Pro1266Leu mutation on structure and function of glycoprotein Ib binding domain of von Willebrand factor","volume":"120","author":"Naqvi","year":"2019","journal-title":"J Cell Biochem"},{"key":"10.1016\/j.jtha.2026.01.023_bib15","doi-asserted-by":"crossref","first-page":"1221","DOI":"10.1182\/blood.2022017239","article-title":"Type 2B von Willebrand disease mutations differentially perturb autoinhibition of the A1 domain","volume":"141","author":"Legan","year":"2023","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2026.01.023_bib16","doi-asserted-by":"crossref","first-page":"1867","DOI":"10.1111\/jth.13775","article-title":"A discontinuous autoinhibitory module masks the A1 domain of von Willebrand factor","volume":"15","author":"Deng","year":"2017","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2026.01.023_bib17","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1182\/blood.2022019577","article-title":"2B or not 2B: art thou autoinhibitory?","volume":"141","author":"McKinnon","year":"2023","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2026.01.023_bib18","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1111\/jth.15528","article-title":"Desialylation of O-glycans activates von Willebrand factor by destabilizing its autoinhibitory module","volume":"20","author":"Voos","year":"2022","journal-title":"J Thromb Haemost"}],"container-title":["Journal of Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S153878362600108X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S153878362600108X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T19:56:56Z","timestamp":1783972616000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S153878362600108X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":18,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["S153878362600108X"],"URL":"https:\/\/doi.org\/10.1016\/j.jtha.2026.01.023","relation":{},"ISSN":["1538-7836"],"issn-type":[{"value":"1538-7836","type":"print"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Caplacizumab resistance in immune thrombotic thrombocytopenic purpura is associated with a von Willebrand factor A1 domain missense variant","name":"articletitle","label":"Article Title"},{"value":"Journal of Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jtha.2026.01.023","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Published by Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.","name":"copyright","label":"Copyright"}]}}