{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T14:18:51Z","timestamp":1781101131757,"version":"3.54.1"},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T00:00:00Z","timestamp":1732665600000},"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":[[2025,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>This multicentre phase II study Fondazione Italiana Linfomi (FIL)\u2010bortezomib plus rituximab plus bendamustine (BRB) tested a combination of bendamustine (90\u2009mg\/m<jats:sup>2<\/jats:sup> on days 1\u20132), rituximab (375\u2009mg\/m<jats:sup>2<\/jats:sup> intravenously on day 1) and bortezomib (1.3\u2009mg\/m<jats:sup>2<\/jats:sup> sc on days 1, 8, 15, 22) every 28\u2009days for six cycles in 38 symptomatic patients with relapsed\/refractory Waldenstrom macroglobulinaemia (RR\u2010WM). Moreover, MYD88<jats:sup>L265P<\/jats:sup> and CXCR4<jats:sup>S338X<\/jats:sup> mutations were tested by droplet digital polymerase chain reaction (ddPCR) both at baseline and at the end of treatment in 21 patients. Overall response rate at the end of therapy was 84.6%, including 4 (11%) complete remission, 15 (39%) very good partial response, 12 (32%) partial responses according to IWWM response criteria. At 18, 24 and 30\u2009months, progression\u2010free survival was 84.2% (95% CI 68.2%\u201392.6%), 81.5% (95%CI 65.1\u201390.7) and 78.8% (95%CI 62.0\u201388.8) respectively. At 18\u2009months, the Overall survival was 92.1% (95%CI 77.5%\u201397.4%). Overall, 19 patients (50%) experienced grade 3\u20134 haematological toxicity, mainly thrombocytopenia, and grade 1\u20133 neuropathy rate was about 10% and required bortezomib dose reduction but did not result in treatment interruption. Moreover, BRB treatment induced the high rates of undetectable molecular minimal residual disease (MRD) at the end of the therapy. BRB regimen used as second line is an effective and well\u2010tolerated salvage treatment for relapsed refractory Waldenstrom macroglobulinaemia patients. MRD monitoring showed promising efficacy in clearing the residual disease.<\/jats:p>","DOI":"10.1111\/bjh.19920","type":"journal-article","created":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T05:50:07Z","timestamp":1732773007000},"page":"556-564","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Efficacy and safety of bendamustine, rituximab and bortezomib treatment in relapsed\/refractory Waldenstrom Macroglobulinaemia: results of phase 2 single\u2010arm <scp>FIL<\/scp>\u2010<scp>BRB<\/scp> trial"],"prefix":"10.1111","volume":"206","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3200-9654","authenticated-orcid":false,"given":"Giulia","family":"Benevolo","sequence":"first","affiliation":[{"name":"AOU Citt\u00e0 della Salute e della Scienza di Torino\u2014Ematologia U  Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Drandi","sequence":"additional","affiliation":[{"name":"Ematologia U, Dipartimento di Biotecnologie Molecolari e Scienze per la Salute Universit\u00e0 di Torino  Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicoletta","family":"Villiv\u00e0","sequence":"additional","affiliation":[{"name":"UOSD di Ematologia, ASL Roma 1  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Castiglione","sequence":"additional","affiliation":[{"name":"SSD Epidemiologia Clinica e Valutativa\u2014AOU Citt\u00e0 della Salute e della Scienza di Torino e CPO  Piemonte Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Monaco","sequence":"additional","affiliation":[{"name":"AOSS Antonio e Biagio e Cesare Arrigo\u2014SC Ematologia  Alessandria Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4155-403X","authenticated-orcid":false,"given":"Carola","family":"Boccomini","sequence":"additional","affiliation":[{"name":"AOU Citt\u00e0 della Salute e della Scienza di Torino\u2014Ematologia  Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Dessi","sequence":"additional","affiliation":[{"name":"Ospedale Businco\u2014SC Ematologia e CTMO  Cagliari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catello","family":"Califano","sequence":"additional","affiliation":[{"name":"Presidio Ospedaliero A. 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