Clinical presentation and management of marginal zone lymphomas

Marginal-zone lymphoma (MZL) includes three subtypes depending on the site of lymphoma involvement: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma); splenic MZL; and nodal MZL. Beside a common cell-of-origin and similarities concerning a possible chronic antigenic stimulation by microbial pathogens and/or autoantigens, the clinical presentation is very different with symptoms related to lymphoma location. MALT and splenic MZL present with an indolent disease with good performance status, no B symptoms, and no adverse prognostic factors and are associated with long survival. Patients with nodal MZL present with a more aggressive disease and have a shorter failure-free survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The optimal treatment has yet to be defined for the three subtypes, and current strategies will be described in this review.