Marginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma (iNHL) with mucosa-associated lymphoid tissue (MALT) lymphoma being the most common subtype. Typical of other iNHLs, a fraction of patients will experience more aggressive disease marked by early relapse despite effective front-line therapy. In this review, we discuss existing prognostic scoring systems for patients with MALT lymphoma undergoing first-line therapy, including the MALT-IPI, the Revised MALT-IPI, the MZL-IPI, and the EMZL-MPI. We review strengths and limitations of these systems and discuss advances in the current understanding of molecular and genomic drivers of lymphomagenesis in this disease, which are not currently reflected in prognostic scoring systems. We review evolving first-line treatment approaches and discuss how there may be opportunities to refine our ability to prognosticate outcomes because of these recent advances. Existing prognostic scoring systems for MALT lymphoma each have their own merit and are valuable for informing risk assessment in both clinical and research contexts, however, as understanding of the molecular and genetic drivers of MALT lymphomas improves and first-line treatment approaches incorporate novel targeted and immune-based therapies, there will likely be opportunities to enhance the current prognostic models.
Sharp et al. (Tue,) studied this question.