Marginal Zone Lymphoma: Factors That Affect the Final Outcome, a Study of 275 Patients

Purpose: Retrospective review and analysis of 275 patients with marginal zone lymphoma (MZL) to determine the prognostic factors that affect outcome in an effort to find a specific prognostic score.

Materials and Methods: Two hundred and seventy five patients with pathologically confirmed MZL were studied. Their median age was 60 years (range 18–89). Male sex was found in 123 patients (45%). Ann Arbor stage I was present in 104 patients (38%), stage II in 27 (10%), stage III in 16 (6%), stage IV in 124 (45%), and unknown in 4 (1%). Most patients were asymptomatic at presentation (90%), with 96% presenting with Zubrod score of 0 or 1. Patients were divided into three groups according to sites of involvement: extranodal (211), nodal (27), and splenic (37). Treatment strategy consisted of: observation in 75 (27%), chemotherapy in 103 (37%), combined chemotherapy and radiation in 24 (9%), and definitive radiation in 71 (26%).

Results: Both univariate and multivariate analysis showed that splenic presentation had a better outcome with a hazard ratio (HR) of 0.14 and p value=0.006, while extranodal presentation with bone marrow involvement had an inferior outcome with HR of 2.27 and p value =0.045. Two factors were found to be associated with decreased overall survival (OS) in the multivariate analysis: Older age (p=0.007) and beta 2 microglobulin >1.8 mg/L (p <0.0001). On the other hand splenic presentation and being asymptomatic at presentation were associated with an improved OS with a p value of 0.002 and 0.017, respectively. Stage at presentation had no impact on the overall survival or disease free survival, and although all patients with splenic MZL were stage IV, these patients had the best outcome. Nodal presentation had a similar survival to extranodal presentation.

Conclusion: Splenic presentation even at advanced stage is associated with a better outcome. Bone marrow involvement in patients with extranodal presentation had a lower OS. Older age, high beta 2 microglobulin, and symptoms at presentation adversely affected the overall survival of patients with MZL.