Nodal marginal zone lymphoma: Clinical features, diagnosis, management and treatment.

Nodular marginal zone lymphoma (NMZL) is a small B-cell lymphoma involving only lymph nodes and is the least common form of MZL constituting about 10% of cases. Patients usually present with advanced disease which must be distinguished from extranodal MZL with lymph node spread. NMZL shares cytological and immunophenotypic features with MALT and splenic MZL, but has a less favorable prognosis than these two categories. It occurs mostly in adults and pediatric cases are rare. Different therapeutic approaches have been used in NMZL, but because of the small patient numbers involved, more definitive treatment is still anticipated. Recent studies suggest that it probably represents a separate entity within the broader indolent lymphoma category. In NMZL there is an emerging need to utilize novel agents, already available for indolent lymphomas. Prospective studies are required to evaluate their therapeutic efficacy for NMZL in the future.