OBJECTIVES : Marginal zone lymphoma (MZL) is a rare, indolent non-Hodgkin lymphoma (NHL) with orphan disease designation. This study aimed to review the global disease burden of MZL and identify current evidence gaps.

METHODS : A systematic literature review was conducted using PubMed, Embase, and selected congress proceedings to identify full-text and conference abstracts between 2016 and 2021. English language studies with an abstract were extracted. Studies were screened and summarized for clinical (incidence, prevalence, mortality, and survival), economic (costs and healthcare resource utilization [HRU]), or humanistic (quality of life, utility, and symptom impact) data.

RESULTS : A total of 48 unique studies (42 clinical, 4 economic, and 2 humanistic) were identified. MZL incidence rate was low in the 2 countries reported with a range of: 0.30 in France to 0.91 in the US (per 100,000 patient-years). Survival and mortality were reported across different MZL subtypes at different disease stages and treatment status in limited countries. 5-year overall survival ranged from 41% (splenic MZL) to 99% (extranodal MZL). Overall rituximab +/- chemotherapy was the most common treatment reported. There is no MZL-related economic burden reported in Europe. In the US, Medicare data suggests HRU increases as the line of therapy advanced. Hospitalization rates ranged from 73% to 86% throughout observed disease course. Two studies (US, Korea) reported humanistic burden of indolent NHL that included MZL. The median Functional Assessment of Cancer Therapy – General (FACT-G) for MZL patients was reported to be 83.3.

CONCLUSIONS : There are limited data focusing specifically on MZL, with the majority reported with other NHL. Epidemiology and clinical burden data skews towards selected developed countries. The economic and humanistic burden of MZL is also vastly underreported globally. Future research is warranted to bridge the evidence gap and understand the impact of heterogenous MZL subtypes on patient outcomes.