• marginal zone lymphoma;
  • mucosa-associated lymphoid tissue;
  • prognostic factors;
  • extranodal;
  • nodal;
  • splenic



A retrospective review and analysis of 275 patients with marginal zone lymphoma (MZL) was performed to determine prognostic factors. An effort was also made to establish a specific prognostic score for patients with extranodal MZL.


Patients were divided into 3 groups according to the type of MZL: extranodal, nodal, and splenic. Factors analyzed included age; gender; presence of B symptoms; Zubrod performance score; clinical stage; serum β2-microglobulin, lactate dehydrogenase, albumin, and hemoglobin levels; and presence of autoimmune disorder.


The 5-year overall survival rates of patients with extranodal, nodal, and splenic MZL were 87%, 89%, and 93%, respectively (P = .95). On multivariate analysis, splenic MZL patients had the best prognosis (hazard ratio, 0.18; P = .018). An elevated serum β2-microglobulin level (P = .010), B symptoms (P = .021), and male gender (P = .036) were found to be correlated with decreased recurrence-free survival (RFS) on multivariate analysis. Using these 3 variables, a 3-tier prognostic scoring system was created for patients with extranodal MZL: low-risk with no adverse factors, intermediate-risk with 1 adverse factor, and high-risk with ≥2 adverse factors. The 5-year RFS rates for the low-risk, intermediate-risk, and high-risk groups were 80%, 71%, and 44%, respectively (P = .01).


Patients with extranodal and nodal MZL have a similar prognosis, whereas patients with splenic MZL have a better prognosis despite the increased prevalence of negative prognostic indicators. With the use of 3 readily available factors, a prognostic scoring system was identified for patients with extranodal MZL. Cancer 2010. © 2010 American Cancer Society.