OBJECTIVES:

Mantle cell lymphoma (MCL), a rare and aggressive disease, accounts for approximately 5% of all B-cell non-Hodgkin’s lymphomas. This review aimed to synthesize the global burden of disease for MCL, which is generally considered to be incurable, by characterizing its epidemiology, natural history, economic, societal, and humanistic burden using real-world evidence (RWE).

METHODS:

Searches were run in EMBASE, Medline, NHSEED, and ECONLit from January 2007–January 2017. Outcomes of interest were incidence, prevalence, quality of life, costs, resource use, mortality, and long-term prognosis. The review was restricted to RWE, selecting cross-sectional or observational studies and reviews of clinical registries. Data from clinical trials were not considered. In total, 1692 abstracts and 44 full-texts were reviewed; 25 studies met the inclusion criteria.

RESULTS:

Standardized MCL incidence rates ranged from 0.1/100,000–1.27/100,000 and varied by sex (female: 0.05/100,000–0.7/100,000, male: 0.18/100,000–1.4/100,000) and geography (0.1/100,000 in Japan, 1.27/100,000 in Denmark). Overall survival (OS) rates of patients at 3 years differed according to age at diagnosis (≤65 years: 76–81%, >65 years: 46–64%) and disease stage (stage I: 73–80%, stage IV: 48–53%), but not by sex. Median OS in patients receiving first-line chemotherapy±rituximab ranged from 27–68 months and in relapsed/refractory (R/R) patients from 4–19 months. Only 1 RWE study reported median progression-free survival for chemotherapy±rituximab (2 months in R/R setting). Median number of hospitalization days with first-line chemotherapy ranged from 0–29 depending on treatment. No quality of life or economic burden data were identified.

CONCLUSIONS:

Our burden of illness review demonstrated that MCL is a rapidly progressing disease with poor outcomes following relapse and low survival rates (age, disease stage, and line of therapy all adversely affected survival). New treatments are needed to improve patient outcomes and reduce the global burden of disease for MCL patients.