Marginal zone lymphomas (MZLs) account for 6% to 9% of all non-Hodgkin lymphomas. Typically MZLs are indolent. However, a subset of MZLs transform into higher-grade lymphomas with aggressive behavior and shorter survival.

Investigators identified risk factors for higher-grade transformation, according to a study published in the Journal of Clinical Oncology. The main predictors of increased higher-grade transformation are failure to achieve clinical remission after initial treatment, elevated lactate dehydrogenase, and more than 4 nodal sites at the time of MZL diagnosis.

The investigators retrospectively evaluated data from 453 patients with biopsy-proven MZL who were seen at Sylvester Comprehensive Cancer Center between 1995 and 2016. A total of 34 patients had biopsy-proven higher-grade transformation (HGT). Seven patients presented with HGT at the time of MZL diagnosis. Median time to HGT occurrence was 29 months for the remaining 27 patients.

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Those in whom HGT occurred within 12 months of diagnosis had a shorter overall survival than both patients with HGT at the time of MZL diagnosis and patients in whom HGT occurred more than 12 months later. Failure to achieve complete remission, higher scores of international prognostic index (IPI), follicular lymphoma IPI, and mucosa-associated lymphoid tissue lymphoma IPI were significant predictors of shorter progression-free survival and shorter overall survival.

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“In our cohort, failure to achieve [clinical remission] after initial treatment was the most significant risk factor for future [higher-grade transformation]. Analysis of lymphoma specimens in patients who achieve [clinical remission] or not after initial MZL treatment may identify genetic changes that predispose to future HGT and should be performed,” noted the authors.


Alderuccio JP, Zhao W, Desai A, et al.Risk factors for transformation to higher-grade lymphoma and its impact on survival in a large cohort of patients with marginal zone lymphoma from a single institution[published online October 12, 2018]. J Clin Oncol.doi: 10.1200/JCO.18.00138