{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:35:40Z","timestamp":1790775340032,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,2,22]],"date-time":"2023-02-22T00:00:00Z","timestamp":1677024000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Myeloid and erythroid precursor vacuolation is a common dysplastic finding associated with myeloid malignancies, toxins, drug, and nutritional deficiencies. It has been described as a core morphologic feature in VEXAS (vacuoles, E1 enzyme, X\u2010linked, autoinflammatory, somatic) syndrome. We sought to determine the number of cases attributable to VEXAS syndrome in bone marrow biopsies and aspirates (BAMB) reporting myeloid precursor vacuolation. We reviewed 1318 individual BAMB reports from January 2020 to July 2021 where \u201cvacuole(s),\u201d \u201cvacuolation,\u201d or \u201cvacuolated\u201d was reported. Bone marrow biopsies with vacuolation confined to blasts or those completed as routine workup prior to stem cell transplant or post induction chemotherapy for AML (acute myeloid leukemia) were excluded. Myeloid and erythroid precursor vacuolation was noted in 219 reports representing 210 patients. The most common etiology was myelodysplastic syndrome (MDS) (38.6%), AML (16.7%), lymphoproliferative disorders and multiple myeloma (7.6%), drug or toxin exposure (5.2%) myeloproliferative neoplasm (MPN) or MPN\/MDS overlap syndrome (4.3%). VEXAS syndrome was determined to be the etiology in 2.9% of patients. Two additional cases of VEXAS syndrome with bone marrow biopsies reported in the specified time frame did not explicitly report myeloid or erythroid precursor vacuolation but were identified based on clinical suspicion and repeat BAMB review. Myeloid and erythroid precursor vacuolation is a dysplastic feature attributable to VEXAS syndrome in at least 2.9% of cases. Standardized reporting of vacuolization, triaging of molecular sequencing and optimal treatment of this disorder are critical issues facing those seeing patients with suspected VEXAS syndrome.<\/jats:p>","DOI":"10.1111\/ejh.13944","type":"journal-article","created":{"date-parts":[[2023,2,15]],"date-time":"2023-02-15T06:49:36Z","timestamp":1676443776000},"page":"633-638","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["<scp>VEXAS<\/scp> syndrome: A review of bone marrow aspirate and biopsies reporting myeloid and erythroid precursor vacuolation"],"prefix":"10.1111","volume":"110","author":[{"given":"Hannah","family":"Cherniawsky","sequence":"first","affiliation":[{"name":"Leukemia\/BMT Program of British Columbia, Vancouver General Hospital  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordan","family":"Friedmann","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hamish","family":"Nicolson","sequence":"additional","affiliation":[{"name":"Department of Pathology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha","family":"Dehghan","sequence":"additional","affiliation":[{"name":"Division of Rheumatology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4718-5212","authenticated-orcid":false,"given":"Ryan J.","family":"Stubbins","sequence":"additional","affiliation":[{"name":"Leukemia\/BMT Program of British Columbia, Vancouver General Hospital  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynda M.","family":"Foltz","sequence":"additional","affiliation":[{"name":"Division of Hematology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather A.","family":"Leitch","sequence":"additional","affiliation":[{"name":"Division of Hematology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gayatri M.","family":"Sreenivasan","sequence":"additional","affiliation":[{"name":"Division of Hematology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberley L. S.","family":"Ambler","sequence":"additional","affiliation":[{"name":"Division of Hematology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas J.","family":"Nevill","sequence":"additional","affiliation":[{"name":"Leukemia\/BMT Program of British Columbia, Vancouver General Hospital  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3360-5471","authenticated-orcid":false,"given":"Eric","family":"McGinnis","sequence":"additional","affiliation":[{"name":"Department of Pathology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorena","family":"Wilson","sequence":"additional","affiliation":[{"name":"National Institutes of Health  Bethesda USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David B.","family":"Beck","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine NYU Grossman School of Medicine  New York USA"},{"name":"Center for Human Genetics and Genomics, NYU Grossman School of Medicine  New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9551-2951","authenticated-orcid":false,"given":"Luke Y. C.","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Hematology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krista M.","family":"Marcon","sequence":"additional","affiliation":[{"name":"Department of Pathology University of British Columbia  Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,2,22]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa2026834"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2022.24836"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11314"},{"issue":"3","key":"e_1_2_10_5_1","first-page":"487","article-title":"Alcohol\u2010induced vacuolization in bone marrow cells: ultrastructure and mechanism of formation","volume":"13","author":"Yeung KY","year":"1988","journal-title":"Blood Cells"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0223013"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.5772\/64422"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1503\/cmaj.211770"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40164\u2010021\u201000217\u20102"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17679"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2021010811"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102016\u201001\u2010643569"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2021.280238"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab524"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmc2102124"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021004749"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17893"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/hs9.0000000000000661"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022016642"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.13944","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ejh.13944","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.13944","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T15:40:12Z","timestamp":1692632412000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.13944"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,22]]},"references-count":18,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,6]]}},"alternative-id":["10.1111\/ejh.13944"],"URL":"https:\/\/doi.org\/10.1111\/ejh.13944","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,22]]},"assertion":[{"value":"2022-12-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-07","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}