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We previously reported that EMZL with multiple mucosal sites (MMS) at diagnosis is characterized by shorter survival. To better recognize patients with different patterns of progression\u2010free survival (PFS) we developed and validated a new prognostic index primarily based on patient's disease characteristics. We derived the \u201cRevised mucosa\u2010associated lymphoid tissue International Prognostic Index\u201d (Revised MALT\u2010IPI) in a large data set (<jats:italic>n<\/jats:italic>\u00a0=\u00a0397) by identifying candidate variables that showed highest prognostic association with PFS. The revised MALT\u2010IPI was validated in two independent cohorts, from the University of Iowa\/Mayo Clinic (<jats:italic>n<\/jats:italic>\u00a0=\u00a0297) and from IELSG\u201019 study (<jats:italic>n<\/jats:italic>\u00a0=\u00a0400). A stepwise Cox regression analysis yielded a model including four independent predictors of shorter PFS. Revised MALT\u2010IPI has scores ranging from 0 to 5, calculated as a sum of one point for each of the following\u2010 age &gt;60\u2009years, elevated LDH, and stage III\u2013IV; and two points for MMS. In the training cohort, the Revised MALT\u2010IPI defined four risk groups: low risk (score 0, reference group), low\u2010medium risk (score 1, HR\u00a0=\u00a01.85, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.008), medium\u2010high risk (score 2, HR\u00a0=\u00a03.84, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.0001), and high risk (score 3+, HR\u00a0=\u00a08.48, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.0001). Performance of the Revised MALT\u2010IPI was similar in external validation cohorts. Revised MALT\u2010IPI is a new index centered on disease characteristics that provides robust risk\u2010stratification identifying a group of patients characterized by earlier progression of disease. Revised MALT\u2010IPI can allow a more disease\u2010adjusted management of patients with EMZL in clinical trials and practice.<\/jats:p>","DOI":"10.1002\/ajh.26715","type":"journal-article","created":{"date-parts":[[2022,9,3]],"date-time":"2022-09-03T18:28:15Z","timestamp":1662229695000},"page":"1529-1537","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Revised <scp>MALT\u2010IPI<\/scp>: A new predictive model that identifies high\u2010risk patients with extranodal marginal zone lymphoma"],"prefix":"10.1002","volume":"97","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2690-3377","authenticated-orcid":false,"given":"Juan Pablo","family":"Alderuccio","sequence":"first","affiliation":[{"name":"Division of Hematology Sylvester Comprehensive Cancer Center  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isildinha M.","family":"Reis","sequence":"additional","affiliation":[{"name":"Department of Public Health Sciences University of Miami Miller School of Medicine  Miami Florida USA"},{"name":"Biostatistics and Bioinformatics Shared Resource Sylvester Comprehensive Cancer Center  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3532-9132","authenticated-orcid":false,"given":"Thomas M.","family":"Habermann","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian K.","family":"Link","sequence":"additional","affiliation":[{"name":"Division of Hematology, Oncology and Bone and Marrow Transplantation University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Thieblemont","sequence":"additional","affiliation":[{"name":"APHP, H\u00f4pital Saint\u2010Louis, Service d'h\u00e9mato\u2010oncologie DMU DHI, Universit\u00e9 de Paris  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annarita","family":"Conconi","sequence":"additional","affiliation":[{"name":"Division of Hematology Ospedale degli Infermi  Biella Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa C.","family":"Larson","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luciano","family":"Cascione","sequence":"additional","affiliation":[{"name":"Clinic of Medical Oncology, Oncology Institute of Southern Switzerland Ente Ospedaliero Cantonale  Bellinzona Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Zhao","sequence":"additional","affiliation":[{"name":"Biostatistics and Bioinformatics Shared Resource Sylvester Comprehensive Cancer Center  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7482-178X","authenticated-orcid":false,"given":"James R.","family":"Cerhan","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Zucca","sequence":"additional","affiliation":[{"name":"Clinic of Medical Oncology, Oncology Institute of Southern Switzerland Ente Ospedaliero Cantonale  Bellinzona Switzerland"},{"name":"Institute of Oncology Research, Faculty of Biomedical Sciences Universit\u00e0 della Svizzera Italiana  Bellinzona Switzerland"},{"name":"Department of Medical Oncology Bern University Hospital and University of Bern  Bern Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9346-9013","authenticated-orcid":false,"given":"Izidore S.","family":"Lossos","sequence":"additional","affiliation":[{"name":"Division of Hematology Sylvester Comprehensive Cancer Center  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,9,19]]},"reference":[{"key":"e_1_2_9_2_1","volume-title":"WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues","author":"Swerdlow 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