Nodal marginal zone lymphoma
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
C83.00 – Small cell B-cell lymphoma, unspecified site
726721002 – Nodal marginal zone B-cell lymphoma
Differential Diagnosis & Pitfalls
- Other B-cell lymphomas – extranodal marginal zone lymphoma, splenic marginal zone lymphoma, mantle cell lymphoma, chronic lymphocytic leukemia, follicular lymphoma
- Lymphoplasmacytic lymphoma
- Splenomegaly can occur with other hematologic disorders including leukemia and other myeloproliferative neoplasms, as well as from nonmalignant causes (autoimmune or infection).