Dual institution experience of nodal marginal zone lymphoma reveals excellent long-term outcomes in the rituximab era.

Abstract

Nodal marginal zone lymphoma (NMZL) is a rare non-Hodgkin lymphoma that arises from mature B-cells. We delineate outcomes, prognostic factors and treatment trends among a large cohort of patients with NMZL in the rituximab era. We identified 56 such patients treated at our institutions. The majority presented with advanced stage disease (78·6%). Over a median follow-up of 38·2months, median progression-free survival (PFS) was 42·4months and median overall survival (OS) was not reached. Kaplan-Meier estimates of OS at 120months after diagnosis was 71·9%. High-risk follicular lymphoma international prognostic index (FLIPI) was associated with inferior PFS. Age >60years and elevated serum lactate dehydrogenase (LDH) were associated with inferior OS. Transformation to diffuse large B-cell lymphoma occurred in 7 patients, 6 of who presented with advanced disease. OS was comparable to our previously reported extranodal MZL cohort. FLIPI score predicted for inferior PFS and OS when both cohorts were analysed together (n=267). In summary, outcomes in NMZL are favourable with a large majority of patients surviving at 120months. High risk FLIPI, age >60years, and elevated serum LDH were associated with inferior outcomes.