{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T15:42:47Z","timestamp":1785944567455,"version":"3.56.0"},"reference-count":16,"publisher":"Wiley","issue":"19","license":[{"start":{"date-parts":[[2022,5,10]],"date-time":"2022-05-10T00:00:00Z","timestamp":1652140800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Intravascular large B\u2010cell lymphoma (lVLBCL) is a very rare type of large B\u2010cell lymphoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a retrospective study on IVLBCL patients treated from 2000 to 2016 in LYSA cooperative group centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010five patients were identified in 23 centers. Median age at diagnosis was 69\u2009years (range 23\u201392). Thirty\u2010four patients (64%) had an IPI score &gt;3 and 40 patients (67%) had a performance status \u22652. The most frequent extra\u2010nodal locations were bone marrow (<jats:italic>n<\/jats:italic>\u00a0=\u200934; 52%), central nervous system (<jats:italic>n<\/jats:italic>\u00a0=\u200925; 39%), and skin (<jats:italic>n<\/jats:italic>\u00a0=\u200921; 33%). Nodal involvement and endocrine system were observed in 34% (<jats:italic>n<\/jats:italic>\u00a0=\u200922) and 18% (<jats:italic>n<\/jats:italic>\u00a0=\u200912) of all cases, respectively. Twenty\u2010six patients (41%) had macrophage activation syndrome. Tumor cells were frequently CD5 positive (52%) with a non\u2010germinal center origin (86%). BCL2 was expressed in 87% of all samples analyzed (<jats:italic>n<\/jats:italic>\u00a0=\u200920) and 43% of patients had a MYC\/BCL2 double expression. Fifty\u2010six patients were treated with a regimen of chemotherapy containing rituximab, among whom 73% reached complete remission. The median progression\u2010free survival (PFS) and median overall survival (OS) were 29.4\u2009months and 63.8\u2009months, respectively. History of autoimmune disorder (Hazard ratio [HR] 3.3 [1.4\u20137.8]; <jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.01), nodal involvement (HR 2.6 [1.4\u20135.1]; <jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.01), lack of anthracycline (HR 0.1 [0\u20130.4] for use; <jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001), or no intensification at first\u2010line regimen (<jats:italic>p<\/jats:italic>\u00a0=\u20090.02) were associated with worse PFS. High\u2010dose methotrexate use was not associated with better PFS or OS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our study highlights the aggressive clinical picture of IVLBCL, in particular the frequency of macrophage activation syndrome, and the need for new therapies despite a response to R\u2010CHOP\u2010like regimen similar to non\u2010intravascular diffuse large B\u2010cell lymphomas.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cam4.4742","type":"journal-article","created":{"date-parts":[[2022,5,11]],"date-time":"2022-05-11T05:13:55Z","timestamp":1652246035000},"page":"3602-3611","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Clinical presentation, outcome, and prognostic markers in patients with intravascular large B\u2010cell lymphoma, a lymphoma study association (LYSA) retrospective study"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6962-3650","authenticated-orcid":false,"given":"Antoine","family":"Bonnet","sequence":"first","affiliation":[{"name":"Service d\u2019h\u00e9matologie clinique, Centre Hospitalier Universitaire Nantes, Nantes, France (at the time of work)  Service d\u2019h\u00e9matologie \u2010 Centre Hospitalier Bretagne Atlantique, Vannes France (now)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Bossard","sequence":"additional","affiliation":[{"name":"Service d'anatomie et cytologie pathologique Centre Hospitalier Universitaire Nantes  Nantes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludovic","family":"Gabellier","sequence":"additional","affiliation":[{"name":"Service d'h\u00e9matologie Centre Hospitalier Universitaire Montpellier  Montpellier 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