{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T20:16:39Z","timestamp":1771532199062,"version":"3.50.1"},"reference-count":39,"publisher":"Wiley","issue":"22","license":[{"start":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T00:00:00Z","timestamp":1732492800000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RC\u20102024\u20102790170"],"award-info":[{"award-number":["RC\u20102024\u20102790170"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>MGRS are new rare clinical entities, whose recognition and optimal management is evolving.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>To implement real\u2010life data, we retrospectively analysed a multicentre cohort of 60 patients with renal biopsy\u2010proven MGRS receiving mainly novel treatments (between 2006 and 2021) in eight Italian centres. Based on renal biopsy, patients were divided into two subgroups: AL amyloidosis (70%, <jats:italic>n<\/jats:italic>\u2009=\u200942) and other\u2010MGRS (30%, <jats:italic>n<\/jats:italic>\u2009=\u200918).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Baseline characteristics follow typical manifestations of MGRS disorders in terms of small clonal burden, laboratory and clinical features. More patients with AL amyloidosis had monotypic lambda light\u2010chain disease, estimated glomerular filtration rate (eGFR) \u2265\u200960 mL\/min and nephrotic proteinuria than other\u2010MGRS group. The most widely used drug was bortezomib, and about one\u2010third of patients underwent ASCT. Overall response rate was 86% with no differences in the two subgroups. However, high\u2010quality hematologic responses\u2009\u2265very good partial response (VGPR) were greater in AL amyloidosis than in other\u2010MGRS group (67% vs 28%, <jats:italic>p<\/jats:italic>\u2009=\u20090.015). The depth of haematological response influenced renal response, obtained in 32 (59%) of evaluable patients, similarly in the subgroups. Indeed, 75% patients with \u2265\u2009VGPR (<jats:italic>p<\/jats:italic>\u2009=\u20090.049) and none with stable disease (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.001) obtained a renal response. No association between renal response and histotypes (<jats:italic>p<\/jats:italic>\u2009=\u20090.9) or type of first\u2010line therapy (<jats:italic>p<\/jats:italic>\u2009=\u20090.3) was found. At a median follow\u2010up of 54.4 months (IQR 24.8\u2013102.8), median progression\u2010free survival (PFS) was 100.1\u2009months (95% CI 34.9\u2013NR), and median overall survival not reached (95% CI 129.8\u2013NR). No significant difference emerged between the two groups in terms of survival outcomes. Achieving \u2265\u2009VGPR was confirmed as the main independent predictor of prolonged PFS in the general population (HR\u2009=\u20090.29, <jats:italic>p<\/jats:italic>\u2009=\u20090.023) and AL amyloidosis group (HR 0.23; <jats:italic>p<\/jats:italic>\u2009=\u20090.023). Preserved renal function at diagnosis was predictive of improved PFS in the AL amyloidosis group (eGFR \u2265\u200960 mL\/min: HR\u2009=\u20090.003; <jats:italic>p<\/jats:italic>\u2009=\u20090.018; eGFR 30\u201360 mL\/min: HR\u2009=\u20090.04, <jats:italic>p<\/jats:italic>\u2009=\u20090.046).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Further research is warranted to develop standardised response criteria and treatment strategies to improve MGRS management.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cam4.70266","type":"journal-article","created":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T06:38:56Z","timestamp":1732603136000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Presentation and Long\u2010Term Survival Outcomes of Patients With Monoclonal Gammopathy of Renal Significance (MGRS): A Multicenter Retrospective Study"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1169-0129","authenticated-orcid":false,"given":"K.","family":"Mancuso","sequence":"first","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna Istituto di Ematologia \u201cSer\u00e0gnoli\u201d  Bologna Italy"},{"name":"Dipartimento di Scienze Mediche e Chirurgiche Universit\u00e0 di Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Mina","sequence":"additional","affiliation":[{"name":"Myeloma Unit, Division of Hematology University of Turin and Azienda Ospedaliero\u2010Universitaria (AOU) Citt\u00e0 della Salute e della Scienza di Torino  Torino Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Rocchi","sequence":"additional","affiliation":[{"name":"IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna Istituto di Ematologia \u201cSer\u00e0gnoli\u201d  Bologna Italy"},{"name":"Dipartimento di Scienze Mediche e Chirurgiche Universit\u00e0 di Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5547-3516","authenticated-orcid":false,"given":"E.","family":"Antonioli","sequence":"additional","affiliation":[{"name":"Hematology Department Careggi Hospital  Florence Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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