Nodal marginal zone lymphoma (NMZL) is a mature postgerminal center low-grade B-cell lymphoma that presents most commonly in peripheral lymph nodes. Peripheral blood and bone marrow involvement can also occur.

NMZL constitutes less than 2% of lymphoid neoplasms and most often occurs in adults with a median age of approximately 60 years old with no sex predominance. Patients with chronic hepatitis C infection are at increased risk. Pediatric nodal marginal zone lymphoma is a distinct entity with marked male predominance and localized disease.

Patients present with local or generalized lymphadenopathy B symptoms (such as fever, weight loss, or night sweats), or they may present with no symptoms at all. Approximately one-third of patients have concomitant extranodal marginal zone lymphoma; therefore, adequate surveillance for extranodal involvement should be performed, especially in patients with history of autoimmune disorders.

Related topics: Extranodal marginal zone lymphoma; Splenic marginal zone lymphoma