{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T03:23:09Z","timestamp":1793330589927,"version":"4.5.0"},"reference-count":36,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Blood Cells, Molecules, and Diseases"],"published-print":{"date-parts":[[2022,3]]},"DOI":"10.1016\/j.bcmd.2021.102636","type":"journal-article","created":{"date-parts":[[2021,11,30]],"date-time":"2021-11-30T14:09:53Z","timestamp":1638281393000},"page":"102636","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":33,"special_numbering":"C","title":["A case of VEXAS syndrome associated with EBV-associated hemophagocytic lymphohistiocytosis"],"prefix":"10.1016","volume":"93","author":[{"given":"Roy L.","family":"Kao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Audrey A.","family":"Jacobsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"Charles J.","family":"Billington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophia L.","family":"Yohe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy K.","family":"Beckman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory M.","family":"Vercellotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.","family":"Pearson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bcmd.2021.102636_bb0005","doi-asserted-by":"crossref","first-page":"2628","DOI":"10.1056\/NEJMoa2026834","article-title":"Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease","volume":"383","author":"Beck","year":"2020","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bcmd.2021.102636_bb0010","doi-asserted-by":"crossref","first-page":"3203","DOI":"10.1182\/bloodadvances.2021004976","article-title":"Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1","volume":"5","author":"Obiorah","year":"2021","journal-title":"Blood Adv."},{"key":"10.1016\/j.bcmd.2021.102636_bb0015","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1136\/annrheumdis-2021-220089","article-title":"Pathogenic UBA1 variants associated with VEXAS syndrome in japanese patients with relapsing polychondritis","volume":"80","author":"Tsuchida","year":"2021","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.bcmd.2021.102636_bb0020","doi-asserted-by":"crossref","first-page":"2163","DOI":"10.1056\/NEJMc2102124","article-title":"Mutant UBA1 and severe adult-onset autoinflammatory disease","volume":"384","author":"Arlet","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bcmd.2021.102636_bb0025","first-page":"1886","article-title":"Somatic mutations in UBA1 define a distinct subset of relapsing polychondritis patients with VEXAS, arthritis","volume":"73","author":"Ferrada","year":"2021","journal-title":"Rheumatol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0030","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s40164-021-00217-2","article-title":"VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder","volume":"10","author":"Huang","year":"2021","journal-title":"Exp. Hematol. Oncol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0035","doi-asserted-by":"crossref","first-page":"3682","DOI":"10.1182\/blood.2020010177","article-title":"Therapeutic options in VEXAS syndrome: insights from a retrospective series","volume":"137","author":"Bourbon","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.bcmd.2021.102636_bb0040","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1016\/S0140-6736(21)01430-6","article-title":"Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: fevers, myalgia, arthralgia, auricular chondritis, and erythema nodosum","volume":"398","author":"Dehghan","year":"2021","journal-title":"Lancet"},{"key":"10.1016\/j.bcmd.2021.102636_bb0045","article-title":"VEXAS syndrome with systemic lupus erythematosus- expanding the spectrum of associated conditions","author":"Sharma","year":"2021","journal-title":"Arthritis Rheumatol. (Hoboken, N.J.)"},{"key":"10.1016\/j.bcmd.2021.102636_bb0050","doi-asserted-by":"crossref","first-page":"2653","DOI":"10.1016\/j.mayocp.2021.06.006","article-title":"Clinical heterogeneity of the VEXAS syndrome: a case series","volume":"96","author":"Koster","year":"2021","journal-title":"Mayo Clin. Proc."},{"key":"10.1016\/j.bcmd.2021.102636_bb0055","first-page":"2613","article-title":"Development and validation of the hscore, a score for the diagnosis of reactive hemophagocytic syndrome, arthritis","volume":"66","author":"Fardet","year":"2014","journal-title":"Rheumatol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0060","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1007\/s10875-021-01070-y","article-title":"A case of VEXAS syndrome complicated by hemophagocytic lymphohistiocytosis","volume":"41","author":"Grey","year":"2021","journal-title":"J. Clin. Immunol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0065","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/fimmu.2021.678927","article-title":"Case report: VEXAS syndrome: from mild symptoms to life-threatening macrophage activation syndrome","volume":"12","author":"Staels","year":"2021","journal-title":"Front. Immunol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0070","doi-asserted-by":"crossref","DOI":"10.3390\/biom11010060","article-title":"The ubiquitin-proteasome system in immune cells","volume":"11","author":"\u00c7etin","year":"2021","journal-title":"Biomolecules."},{"key":"10.1016\/j.bcmd.2021.102636_bb0075","doi-asserted-by":"crossref","first-page":"1783","DOI":"10.3390\/jcm10081783","article-title":"Role of proteasomes in inflammation","volume":"10","author":"Goetzke","year":"2021","journal-title":"J. Clin. Med."},{"key":"10.1016\/j.bcmd.2021.102636_bb0080","doi-asserted-by":"crossref","DOI":"10.1053\/j.seminhematol.2021.10.004","article-title":"Clonal hematopoiesis and VEXAS syndrome: survival of the fittest clones?","author":"Kusne","year":"2021","journal-title":"Semin. Hematol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0085","article-title":"Successful allogeneic hematopoietic stem cell transplantation in patients with VEXAS syndrome: a two center experience","author":"Diarra","year":"2021","journal-title":"Blood Adv."},{"key":"10.1016\/j.bcmd.2021.102636_bb0090","article-title":"UBA1 and DNMT3A mutations in VEXAS syndrome. A case report and literature review","author":"Shaukat","year":"2021","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"10.1016\/j.bcmd.2021.102636_bb0095","first-page":"1","article-title":"Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American consortium for histiocytosis (NACHO)","volume":"66","author":"Jordan","year":"2019","journal-title":"pediatr. BloodCancer"},{"key":"10.1016\/j.bcmd.2021.102636_bb0100","doi-asserted-by":"crossref","first-page":"2465","DOI":"10.1182\/blood.2018894618","article-title":"Recommendations for the management of hemophagocytic lymphohistiocytosis in adults","volume":"133","author":"La Ros\u00e9e","year":"2019","journal-title":"Blood"},{"key":"10.1016\/j.bcmd.2021.102636_bb0105","first-page":"2717","article-title":"Hyperinflammation: on the pathogenesis and treatment of macrophage activation syndrome","volume":"110","author":"Andersson","year":"2021"},{"key":"10.1016\/j.bcmd.2021.102636_bb0110","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1016\/S0140-6736(13)61048-X","article-title":"Adult haemophagocytic syndrome","volume":"383","author":"Ramos-Casals","year":"2014","journal-title":"Lancet"},{"key":"10.1016\/j.bcmd.2021.102636_bb0115","doi-asserted-by":"crossref","first-page":"269","DOI":"10.3324\/haematol.2018.198655","article-title":"Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy","volume":"104","author":"Yoon","year":"2019","journal-title":"Haematologica"},{"key":"10.1016\/j.bcmd.2021.102636_bb0120","first-page":"e358","article-title":"Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome","volume":"2","author":"Mehta","year":"2020","journal-title":"LancetRheumatol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0125","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.614704","article-title":"Use of the JAK inhibitor ruxolitinib in the treatment of hemophagocytic lymphohistiocytosis","volume":"12","author":"Keenan","year":"2021","journal-title":"Front. Immunol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0130","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1182\/blood.2020007930","article-title":"JAK inhibition for murine HLH requires complete blockade of IFNg signaling and is limited by toxicity of JAK2 inhibition","volume":"138","author":"Chaturvedi","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.bcmd.2021.102636_bb0135","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1111\/bjh.12386","article-title":"Treatment of epstein barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens","volume":"162","author":"Chellapandian","year":"2013","journal-title":"Br. J. Haematol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0140","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1182\/blood.2019003886","article-title":"Nivolumab treatment of relapsed\/refractory epstein-barr virus-associated hemophagocytic lymphohistiocytosis in adults","volume":"135","author":"Liu","year":"2020","journal-title":"Blood"},{"key":"10.1016\/j.bcmd.2021.102636_bb0145","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1182\/bloodadvances.2018025858","article-title":"Treatment of refractory hemophagocytic lymphohistiocytosis with emapalumab despite severe concurrent infections","volume":"3","author":"Lounder","year":"2019","journal-title":"Blood Adv."},{"key":"10.1016\/j.bcmd.2021.102636_bb0150","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1002\/ajh.24976","article-title":"Ruxolitinib-associated infections: a systematic review and meta-analysis","volume":"93","author":"Lussana","year":"2018","journal-title":"Am. J. Hematol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0155","first-page":"E356","article-title":"A case of polychondritis-onset refractory organizing pneumonia with cytopaenia diagnosed as VEXAS syndrome: the disease course of 7 years","volume":"60","author":"Sakuma","year":"2021","journal-title":"Rheumatol. (United Kingdom)"},{"key":"10.1016\/j.bcmd.2021.102636_bb0160","first-page":"1896","article-title":"Extensive multiple organ involvement in VEXAS syndrome, arthritis","volume":"73","author":"Takahashi","year":"2021","journal-title":"Rheumatol."},{"key":"10.1016\/j.bcmd.2021.102636_bb0165","doi-asserted-by":"crossref","first-page":"6469","DOI":"10.1021\/acs.jmedchem.9b00101","article-title":"Proteasome activation as a new therapeutic approach to target proteotoxic disorders","volume":"62","author":"Njomen","year":"2019","journal-title":"J. Med. Chem."},{"key":"10.1016\/j.bcmd.2021.102636_bb0170","unstructured":"A Phase II Study of Allogeneic Hematopoietic Stem Cell Transplant for Subjects With VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) Syndrome., Clin. Identifier NCT05027945. (n.d.). https:\/\/clinicaltrials.gov\/ct2\/show\/NCT05027945 (accessed October 13, 2021)."},{"key":"10.1016\/j.bcmd.2021.102636_bb0175","first-page":"3591","article-title":"VEXAS syndrome","volume":"137","author":"Grayson","year":"2021","journal-title":"Blood"},{"key":"10.1016\/j.bcmd.2021.102636_bb0180","doi-asserted-by":"crossref","first-page":"2731","DOI":"10.1038\/s41375-021-01353-8","article-title":"Prevalence of UBA1 mutations in MDS\/CMML patients with systemic inflammatory and auto-immune disease","volume":"35","author":"Zhao","year":"2021","journal-title":"Leukemia"}],"container-title":["Blood Cells, Molecules, and Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1079979621001029?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1079979621001029?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T12:36:17Z","timestamp":1761050177000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1079979621001029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3]]},"references-count":36,"alternative-id":["S1079979621001029"],"URL":"https:\/\/doi.org\/10.1016\/j.bcmd.2021.102636","relation":{},"ISSN":["1079-9796"],"issn-type":[{"value":"1079-9796","type":"print"}],"subject":[],"published":{"date-parts":[[2022,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"A case of VEXAS syndrome associated with EBV-associated hemophagocytic lymphohistiocytosis","name":"articletitle","label":"Article Title"},{"value":"Blood Cells, Molecules, and Diseases","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bcmd.2021.102636","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}],"article-number":"102636"}}