Revised MALT-IPI: A new predictive model that identifies high-risk patients with extranodal marginal zone lymphoma

Juan Pablo Alderuccio, Isildinha M. Reis, Thomas M. Habermann, Brian K. Link, Catherine Thieblemont, Annarita Conconi, Melissa C. Larson, Luciano Cascione, Wei Zhao, James R. Cerhan, Emanuele Zucca, Izidore S. Lossos

Research output: Contribution to journalArticlepeer-review


Extranodal marginal zone lymphoma (EMZL) is a heterogeneous disease with a subset of patients exhibiting a more aggressive course. 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-free survival (PFS) we developed and validated a new prognostic index primarily based on patient's disease characteristics. We derived the “Revised mucosa-associated lymphoid tissue International Prognostic Index” (Revised MALT-IPI) in a large data set (n = 397) by identifying candidate variables that showed highest prognostic association with PFS. The revised MALT-IPI was validated in two independent cohorts, from the University of Iowa/Mayo Clinic (n = 297) and from IELSG-19 study (n = 400). A stepwise Cox regression analysis yielded a model including four independent predictors of shorter PFS. Revised MALT-IPI has scores ranging from 0 to 5, calculated as a sum of one point for each of the following- age >60 years, elevated LDH, and stage III–IV; and two points for MMS. In the training cohort, the Revised MALT-IPI defined four risk groups: low risk (score 0, reference group), low-medium risk (score 1, HR = 1.85, p =.008), medium-high risk (score 2, HR = 3.84, p <.0001), and high risk (score 3+, HR = 8.48, p <.0001). Performance of the Revised MALT-IPI was similar in external validation cohorts. Revised MALT-IPI is a new index centered on disease characteristics that provides robust risk-stratification identifying a group of patients characterized by earlier progression of disease. Revised MALT-IPI can allow a more disease-adjusted management of patients with EMZL in clinical trials and practice.

Original languageEnglish (US)
Pages (from-to)1529-1537
Number of pages9
JournalAmerican journal of hematology
Issue number12
StatePublished - Dec 2022

ASJC Scopus subject areas

  • Hematology


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