{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T18:25:04Z","timestamp":1791570304965,"version":"4.3.4"},"reference-count":32,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2019,3,10]],"date-time":"2019-03-10T00:00:00Z","timestamp":1552176000000},"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":["American J Hematol"],"published-print":{"date-parts":[[2019,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Between 11 and 37% of extranodal marginal zone lymphoma (EMZL) patients present with disease involvement in multiple mucosal sites (MMS). We analyzed 405 EMZL patients seen between 1995 and 2017: 265 (65.4%) patients presented with stage I disease, 49 of 309 (15.8%) patients with bone marrow involvement, and 35 of 328 (10.7%) patients with monoclonal gammopathy (MG). Forty\u2010three (10.6%) patients had MMS presentation, which was more frequently seen in patients aged &gt;60\u2009years (55.8%). Five (17.9%) of 28 MMS patients had MG. MMS patients commonly exhibited the International Prognostic Index (IPI) &gt;2 (79.1%), Follicular Lymphoma International Prognostic Index (FLIPI) &gt;2 (39.5%), and Mucosa\u2010Associated Lymphoid Tissue Lymphoma International Prognostic Index (MALT\u2010IPI) 2\u20103 (60.5%). Both MMS presentation and MG were associated with shorter survival univariately. In multivariable Cox regression models, shorter progression\u2010free survival (PFS) and overall survival (OS) were observed in patients with MMS (hazard ratio [HR]\u2009=\u20093.08 and 2.92, respectively), age\u2009\u226560\u2009years (HR\u2009=\u20091.52 and 2.45, respectively), and in patients who failed to attain a complete remission following initial therapy (HR\u2009=\u20093.27 and 2.13, respectively). Elevated lactate dehydrogenase was associated with shorter PFS (HR\u2009=\u20091.92), while anemia (HR\u2009=\u20092.46) was associated with shortened OS. MALT\u2010IPI \u22652 (HR\u2009=\u20092.47 and 4.75), FLIPI &gt;2 (HR\u2009=\u20091.65 and 2.09), and IPI &gt;2 (HR\u2009=\u20092.09 and 1.73) were associated with shorter PFS and OS, respectively. Higher grade transformation (HGT) occurred in 11 (25.6%) MMS patients with a 5\u2010year cumulative incidence of 13.2% (95% CI 4.7\u201026.1%). EMZL patients with MMS presentation represent a novel clinical subset associated with shorter PFS, OS, and higher incidence of HGT that needs novel therapeutic approaches.<\/jats:p>","DOI":"10.1002\/ajh.25446","type":"journal-article","created":{"date-parts":[[2019,2,20]],"date-time":"2019-02-20T05:37:51Z","timestamp":1550641071000},"page":"585-596","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Short survival and frequent transformation in extranodal marginal zone lymphoma with multiple mucosal sites presentation"],"prefix":"10.1002","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2690-3377","authenticated-orcid":false,"given":"Juan Pablo","family":"Alderuccio","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine University of Miami, Miller School of Medicine  Miami Florida"},{"name":"Sylvester Comprehensive Cancer Center University of Miami, Miller School of 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I.","family":"de la Fuente","sequence":"additional","affiliation":[{"name":"Sylvester Comprehensive Cancer Center University of Miami, Miller School of Medicine  Miami Florida"},{"name":"Division of Neuro\u2010Oncology, Department of Neurology University of Miami, Miller School of Medicine  Miami Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad","family":"Husnain","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine University of Miami, Miller School of Medicine  Miami Florida"},{"name":"Sylvester Comprehensive Cancer Center University of Miami, Miller School of Medicine  Miami Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph D.","family":"Rosenblatt","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine University of Miami, Miller School of Medicine  Miami Florida"},{"name":"Sylvester Comprehensive Cancer Center University of Miami, Miller 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