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Marginal Zone B-Cell Lymphoma: A Clinical Comparison of Nodal and Mucosa-Associated Lymphoid Tissue Types

From the University of Southern California (USC) and Los Angeles County+USC Healthcare Network, Los Angeles, CA; University of Nebraska Medical Center, Omaha, NE; Ospedale San Giovanni, Bellinzona, Switzerland; Hotel Dieu de Paris, Paris, France; Harvard Medical School, Boston, MA; St James University, Leeds, United Kingdom; and University of Würzburg, Würzburg, Germany.

Address reprint requests to Bharat N. Nathwani, MD, Los Angeles County–University of Southern California Healthcare Network, General Hospital, Rm 2422, 1200 North State St, Los Angeles, CA 90033.

PURPOSE: In the International Lymphoma Study Group classification of lymphoma, extranodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) type is listed as a distinctive entity. However, nodal MZL is listed as a provisional entity because of questions as to whether it is truly a disease or just an advanced stage of MALT-type MZL. To resolve the issue of whether primary nodal MZL without involvement of mucosal sites exists and whether it is clinically different from extranodal MALT-type lymphoma, we compared the clinical features of these two lymphomas.

PATIENTS AND METHODS: Five expert hematopathologists reached a consensus diagnosis of MZL in 93 patients. Seventy-three were classified as having MALT-type MZL because of involvement of a mucosal site at the time of diagnosis, and 20 were classified as having nodal MZL because of involvement of lymph nodes without involvement of a mucosal site.

RESULTS: A comparison of the clinical features of nodal MZL and MALT-type MZL showed that more patients with nodal MZL presented with advanced-stage disease (71% v 34%; P = .02), peripheral lymphadenopathy (100% v 8%; P < .001), and para-aortic lymphadenopathy (56% v 14%; P < .001) than those with MALT-type MZL. However, fewer patients with nodal MZL had a large mass ( 5 cm) than those with MALT-type MZL (31% v 68%; P = .03). The 5-year overall survival of patients with nodal MZL was lower than that for patients with MALT-type MZL (56% v 81%; P = .09), with a similar result for failure-free survival (28% v 65%; P = .01). Comparisons of patients with International Prognostic Index scores of 0 to 3 showed that those with nodal MZL had lower 5-year overall survival (52% v 88%; P = .025) and failure-free survival (30% v 75%; P = .007) rates than those with MALT-type MZL.

CONCLUSION: Nodal MZL seems to be a distinctive disease entity rather than an advanced stage of MALT-type MZL because the clinical presentations and survival outcomes are different in these two types of MZL. Clinically, nodal MZL is similar to other low-grade, node-based B-cell lymphomas, such as follicular and small lymphocytic lymphomas.

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