{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T20:18:41Z","timestamp":1788034721850,"version":"build-2784847793"},"reference-count":33,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T00:00:00Z","timestamp":1786924800000},"content-version":"vor","delay-in-days":16,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","award":["2017WXR7ZT"],"award-info":[{"award-number":["2017WXR7ZT"]}],"id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["HemaSphere"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Thrombotic events (TEs) occur in up to 40% of patients with vacuoles, E1 enzyme, X\u2010linked, autoinflammatory, and somatic (VEXAS) syndrome, but data on its clinical\u2010genomics features and anticoagulation strategies are limited. To gain more insight into this, we conducted a two\u2010step study evaluating the prevalence and outcome of TE in VEXAS. First, among 1086 patients followed for TEs, 198 were men aged &gt;40 years with unprovoked thrombosis and no known thrombophilia; 21 also had at least one VEXAS\u2010compatible feature and underwent\n                    <jats:italic>UBA1<\/jats:italic>\n                    exon 3 testing. No\n                    <jats:italic>UBA1<\/jats:italic>\n                    mutation was detected in these 21 patients. Next, we leveraged our Italian VEXAS network, and we accrued 87 molecularly confirmed Italian VEXAS cases (median age 70 years). Any history of TE was documented in 43\/87 patients (49%), deep vein thrombosis being the most common (71%). Because follow\u2010up varied, incident thrombosis was analyzed using a time\u2010to\u2010first\u2010event framework from molecular VEXAS diagnosis, with death without prior TE treated as a competing event. Among 49 patients without prior\/concomitant TE, five developed incident post\u2010diagnosis TE; the 24\u2010month cumulative incidence was 18.3%. Thrombophilia testing revealed a 15% co\u2010occurrence, including heterozygous Factor V Leiden, Factor II G20210A, and anti\u2010cardiolipin antibodies. Treatments comprised direct oral anticoagulants (DOACs) (51%), low molecular weight heparin (LMWH) (28%), Fondaparinux (14%), and vitamin K antagonists (AVKs) (7%). Notably, 27% experienced multiple TEs, of which 22% occurring despite anticoagulation during disease flares. Our findings provide an updated cartography of VEXAS\u2010related TE, suggesting early screening for thrombophilia in these patients to inform both personalized anticoagulation and disease\u2010control strategies.\n                  <\/jats:p>","DOI":"10.1002\/hem3.70451","type":"journal-article","created":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T12:26:36Z","timestamp":1786969596000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An Italian cartography of VEXAS\u2010related thrombosis"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-3431-7333","authenticated-orcid":false,"given":"Giorgia","family":"Ranucci","sequence":"first","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"Pascale","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittorio","family":"Forte","sequence":"additional","affiliation":[{"name":"Clinical Biochemistry Department of Laboratory Medicine University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4994-7719","authenticated-orcid":false,"given":"Elisa","family":"Diral","sequence":"additional","affiliation":[{"name":"Unit of Hematology and Bone Marrow Transplantation IRCCS San Raffaele Hospital Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrado","family":"Campochiaro","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) IRCCS Ospedale San Raffaele &amp; Vita\u2010Salute San Raffaele University Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Unit of Molecular Hematology &amp; Precision Medicine Fondazione IRCCS Policlinico San Matteo Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariachiara","family":"Cuccaro","sequence":"additional","affiliation":[{"name":"Hematology Unit Azienda Ospedaliero Universitaria Senese, University of Siena Siena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Cattaneo","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and Department of Clinical and Experimental Sciences University of Brescia Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Crisafulli","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and Department of Clinical and Experimental Sciences University of Brescia Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Cardillo","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Renola","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Meddi","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianna","family":"Velocci","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Casciani","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Romano","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pellegrino","family":"Musto","sequence":"additional","affiliation":[{"name":"Department of Precision and Regenerative Medicine and Ionian Area \u201cAldo Moro\u201d University Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serafina","family":"Barbato","sequence":"additional","affiliation":[{"name":"Hematology, Department of Clinical Medicine and Surgery University of Naples \u201cFederico II\u201d Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0695-3879","authenticated-orcid":false,"given":"Giorgia","family":"Battipaglia","sequence":"additional","affiliation":[{"name":"Hematology, Department of Clinical Medicine and Surgery University of Naples \u201cFederico II\u201d Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Frigeni","sequence":"additional","affiliation":[{"name":"Hematology and Bone Marrow Transplant Unit ASST Papa Giovanni XXIII Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika","family":"Morsia","sequence":"additional","affiliation":[{"name":"Hematology Department Azienda Ospedaliera Universitaria Ospedali Riuniti di Ancona Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Savi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences University of Turin Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Papayannidis","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna Istituto di Ematologia \u201cSer\u00e0gnoli\u201d Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cris\u00e0","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Translation Medicine Universit\u00e0 del Piemonte Orientale and Azienda Ospedaliero\u2010Universitaria Maggiore della Carit\u00e0 Novara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Cicconi","sequence":"additional","affiliation":[{"name":"Department of Hematology Polo Universitario Pontino, S.M. 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