{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T11:57:32Z","timestamp":1785585452049,"version":"3.56.0"},"reference-count":50,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T00:00:00Z","timestamp":1781049600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T00:00:00Z","timestamp":1781049600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Acquired von Willebrand Syndrome (AVWS) is a rare bleeding disorder characterized by quantitative or qualitative defects of von Willebrand factor (VWF) in patients without a personal or family history of bleeding. It is frequently associated with systemic diseases, particularly lymphoproliferative disorders (LPDs) and myeloproliferative neoplasms (MPNs). In this single\u2010center, retrospective cross\u2010sectional study, we included patients diagnosed with AVWS at the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center between April 2014 and March 2025. Bleeding severity was assessed using the ISTH\u2010BAT score. Laboratory tests included FVIII:C, VWF:Ag, VWF:GPIbR, VWF:RCo, VWF:CB, VWFpp, and multimer analysis. Among 140 patients, 106 (76%) had MPNs and 26 (19%) LPDs. At least one bleeding symptom was observed in 70% of patients, with clinically significant bleeding occurring in 24% of the cohort. Clinically relevant bleeding (mainly mucocutaneous and gastrointestinal) was more frequent and severe in LPDs (58%) than in MPNs (13%). LPDs showed severe VWF functional defects, marked HMWM loss, and elevated VWFpp\/VWF:Ag ratios (median 6.7), consistent with accelerated clearance. MPNs displayed mild HMWM reduction, normal clearance (median VWFpp\/VWF:Ag ratio 1.0), and an inverse correlation between platelet count and the degree of HMWM depletion (\u03c1\u2009=\u2009\u22120.48,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). Bleeding severity correlated inversely with VWF:GPIbR in LPDs (\u03c1\u2009=\u2009\u22120.50,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.02) and with VWF:RCo in MPNs. Anti\u2010VWF antibodies were found in 30% of tested LPDs or autoimmune cases. The two main phenotypes presented in AVWS were immune\u2010mediated in LPDs and platelet\u2010mediated in MPNs. Understanding the underlying mechanism is crucial for accurate diagnosis and targeted treatment to reduce bleeding risk and improve outcomes.\n                  <\/jats:p>","DOI":"10.1002\/ajh.70399","type":"journal-article","created":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T06:15:34Z","timestamp":1781158534000},"page":"2147-2156","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical and Laboratory Characterization of Acquired Von Willebrand Syndrome"],"prefix":"10.1002","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2297-5231","authenticated-orcid":false,"given":"Alessandro","family":"Ciavarella","sequence":"first","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1186-6005","authenticated-orcid":false,"given":"Luciano","family":"Baronciani","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6630-7879","authenticated-orcid":false,"given":"Omid","family":"Seidizadeh","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4180-6011","authenticated-orcid":false,"given":"Paola","family":"Colpani","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elio","family":"Ingenito","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7571-023X","authenticated-orcid":false,"given":"Daniele","family":"Cattaneo","sequence":"additional","affiliation":[{"name":"Hematology Division Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"},{"name":"Department of Oncology and Hemato\u2010Oncology University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0778-2271","authenticated-orcid":false,"given":"Maria Teresa","family":"Pagliari","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4187-4962","authenticated-orcid":false,"given":"Addolorata","family":"Truma","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4401-0812","authenticated-orcid":false,"given":"Alessandra","family":"Iurlo","sequence":"additional","affiliation":[{"name":"Hematology Division Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8456-7519","authenticated-orcid":false,"given":"Simona Maria","family":"Siboni","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7423-9864","authenticated-orcid":false,"given":"Flora","family":"Peyvandi","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"},{"name":"Department of Pathophysiology and Transplantation Universit\u00e0 degli Studi di Milano  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,10]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(11)62333-1"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102002\u201027824"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1619606"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2022.08.025"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/HS9.0000000000000586"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2005.11.003"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049\u20103848(12)70003\u20106"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:THRO.0000011369.70824.e6"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V64.3.614.614"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102006\u2010949663"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277\u2010016\u20102650\u2010x"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365\u20102516.2003.00750.x"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100038\u20101660481"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtha.2023.01.012"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejim.2016.11.011"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2020.255117"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.3109\/10428199609074364"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102007\u2010996122"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12967"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538\u20107836.2009.03640.x"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa022831"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538\u20107836.2011.04247.x"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep28563"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1677\/joe.0.1540231"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12275"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.12657"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277\u2010002\u20100483\u20102"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH10\u201002\u20100134"},{"key":"e_1_2_12_30_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V64.5.981.981"},{"issue":"3","key":"e_1_2_12_31_1","first-page":"264","article-title":"Acquired von Willebrand Factor Abnormalities in Myeloproliferative Disorders and Other Hematologic Diseases: A Retrospective Analysis by a Single Institution","volume":"87","author":"S\u00e1nchez\u2010Luceros A.","year":"2002","journal-title":"Haematologica"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1182\/hematology.2019000066"},{"issue":"2","key":"e_1_2_12_33_1","first-page":"345","article-title":"Acquired von Willebrand Syndrome: Data From an International Registry","volume":"84","author":"Federici A. B.","year":"2000","journal-title":"Thrombosis and Haemostasis"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100041\u20101730818"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1055\/a\u20101806\u20109972"},{"issue":"1","key":"e_1_2_12_36_1","first-page":"44","article-title":"The Role of the von Willebrand Factor Collagen\u2010Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36\u2010Year History","volume":"50","author":"Favaloro E. J.","year":"2024","journal-title":"Seminars in Thrombosis and Hemostasis"},{"key":"e_1_2_12_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2014.09.020"},{"key":"e_1_2_12_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03975.x"},{"key":"e_1_2_12_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12503"},{"key":"e_1_2_12_40_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2022007216"},{"key":"e_1_2_12_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.70060"},{"key":"e_1_2_12_42_1","first-page":"77","article-title":"A Sensitive Ristocetin Co\u2010Factor Activity Assay with Recombinant Glycoprotein Iba for The diagnosis of Patients With Low von Willebrand factor Levels","volume":"89","author":"Federici A. B.","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_12_43_1","doi-asserted-by":"publisher","DOI":"10.1002\/rth2.12141"},{"key":"e_1_2_12_44_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100041\u20101741038"},{"key":"e_1_2_12_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102010\u201011\u2010297580"},{"key":"e_1_2_12_46_1","doi-asserted-by":"publisher","DOI":"10.5045\/br.2023.2022218"},{"issue":"3","key":"e_1_2_12_47_1","first-page":"264","article-title":"Acquired von Willebrand Factor Abnormalities in Myeloproliferative disorders and Other Hematologic Diseases: A Retrospective Analysis by a Single institution","volume":"87","author":"S\u00e1nchez\u2010Luceros A.","year":"2002","journal-title":"Haematologica"},{"key":"e_1_2_12_48_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12865\u2010017\u20100213\u20100"},{"key":"e_1_2_12_49_1","doi-asserted-by":"publisher","DOI":"10.20452\/pamw.3141"},{"key":"e_1_2_12_50_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15717"},{"key":"e_1_2_12_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtha.2023.12.009"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.70399","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ajh.70399","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.70399","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T11:19:22Z","timestamp":1785583162000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.70399"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,10]]},"references-count":50,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2026,9]]}},"alternative-id":["10.1002\/ajh.70399"],"URL":"https:\/\/doi.org\/10.1002\/ajh.70399","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,10]]},"assertion":[{"value":"2026-02-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-21","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}