{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T03:07:52Z","timestamp":1790564872277,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,10,14]],"date-time":"2021-10-14T00:00:00Z","timestamp":1634169600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2022,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Azacitidine can be effective in myelodysplastic syndromes (MDS) associated with inflammatory\/autoimmune diseases. Vacuoles, E1 Enzyme, X\u2010linked, Autoinflammatory, Somatic syndrome (VEXAS) is a new monogenic autoinflammatory syndrome caused by somatic ubiquitin\u2010like modifier\u2010activating enzyme 1 (<jats:italic>UBA1<\/jats:italic>) mutation, often associated with MDS, whose treatment is difficult and not yet codified. Based on a French nationwide registry of 116 patients with VEXAS, we report the efficacy and safety of azacitidine treatment in 11 patients with VEXAS with MDS. Clinical response of VEXAS to azacitidine was achieved in five patients (46%), during 6, 8+, 12, 21, 27+ months respectively, suggesting that azacitidine can be effective in selected patients with VEXAS and associated MDS.<\/jats:p>","DOI":"10.1111\/bjh.17893","type":"journal-article","created":{"date-parts":[[2021,10,17]],"date-time":"2021-10-17T15:19:26Z","timestamp":1634483966000},"page":"969-974","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":153,"title":["Azacitidine for patients with Vacuoles, E1 Enzyme, X\u2010linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry"],"prefix":"10.1111","volume":"196","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6891-9238","authenticated-orcid":false,"given":"Thibault","family":"Comont","sequence":"first","affiliation":[{"name":"Department of Internal Medicine IUCT\u2010Oncopole University Hospital of Toulouse, Universit\u00e9 Toulouse III \u2013 Paul Sabatier Toulouse France <!--Query ID=\"q6\" Text=\"AUTHOR: Please check that authors and affiliations have been set correctly.\"-->"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1682-8657","authenticated-orcid":false,"given":"Mael","family":"Heiblig","sequence":"additional","affiliation":[{"name":"Department of Hematology, Lyon\u2010Sud Hospital, Hospices Civils de Lyon Universit\u00e9 Lyon Sud Pierre B\u00e9nite France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Rivi\u00e8re","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University of Bordeaux Haut\u2010L\u00e9v\u00eaque Hospital Pessac France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Terriou","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology University of Lille CHU Lille Lille 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremie","family":"Dion","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine IUCT\u2010Oncopole University Hospital of Toulouse, Universit\u00e9 Toulouse III \u2013 Paul Sabatier Toulouse France <!--Query ID=\"q6\" Text=\"AUTHOR: Please check that authors and affiliations have been set correctly.\"-->"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Kosmider","sequence":"additional","affiliation":[{"name":"Hematology laboratory, H\u00f4pital Cochin, Assistance Publique\u2010H\u00f4pitaux de Paris (APH\u2010HP) Universit\u00e9 de Paris Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Terrier","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, H\u00f4pital Cochin, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP) Universit\u00e9 de Paris Paris 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