Nodal Marginal Zone Lymphoma

DISEASE FEATURES Pathology

In contrast with mucosa-based extranodal MALT lymphoma, nodal MZL is typically lymph node based. The tumor cell morphology is heterogeneous and resembles the lymph node involvement of extranodal and splenic MZL. The marginal zone and interfollicular areas are infiltrated by marginal zone B cells, monocy-toid B cells, or small B lymphocytes. Plasma cell differentiation can be present, as well as some large cells.3,117-120

Genetic abnormalities

No specific genomic alteration is known to occur in nodal MZL. The most common alterations, such as gain of 3q, are also present in extranodal and splenic MZL.

Analysis of the IgH genes suggest a prevalence of cases with mutated IgH genes, but, similarly to splenic MZL, unmutated cases do exist.121-123 These data are in accordance with the different normal B-cell populations resident within the marginal zone, which comprise both naive and postgerminal center B cells.124 As noted, both nodal and splenic MZL have been associated with HCV infection.97,98,100,101,125-127

Clinical features

The clinical data are sparse and have been largely drawn from pathologic series rather than clinical cen-ters.128'129 Nodal MZL is a disease of older people, with median age at presentation in the sixth decade, and affects both sexes, with an unusual (albeit slight)

female predominance. The most common presenting feature is a localized adenopathy, most often in the neck.

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