Clinical Presentation and Management of Marginal Zone Lymphomas

Abstract

Marginal-zone lymphoma (MZL) includes three subtypes depending on the site of lymphoma involvement: extranodal marginal zone B-cell lymphoma of mucosaassociated lymphoid tissue (MALT-lymphoma); splenic MZL; and nodal MZL. Beside a common cell-of-origin and similarities concerning a possible chronic antigenic stimulation by microbial pathogens and/or autoantigens, the clinical presentation is very different with symptoms related to lymphoma location. MALT and splenic MZL present with an indolent disease with good performance status, no B symptoms, and no adverse prognostic factors and are associated with long survival. Patients with nodal MZL present with a more aggressive disease and have a shorter failurefree survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The optimal treatment has yet to be defined for the three subtypes, and current strategies will be described in this review.Marginal zone B-cell lymphomas (MZL) represent a group of lymphomas whose cells originate from B lymphocytes normally present in a distinct anatomical location, the socalled "marginal zone" (MZ) of the secondary lymphoid follicles.1 These cells are anatomically localized in the lymphoid organs (spleen and lymph nodes) and in the nonlymphoid organs (mucosa-associated lymphoid tissue [MALT] or non-mucosal tissue such as skin, orbit and dura). Depending on the site of involvement, the International Lymphoma Study Group individualized three distinct subtypes of MZL: (1) extranodal MZL of MALT type, (2) splenic MZL (with or without villous lymphocytes), and (3) nodal MZL (with or without monocytoid B cells).2,3 Despite this classification, the relative rarity of these lymphomas and difficulties in distinguishing them from other low-grade lymphoma subtypes are obstacles to conducting epidemiological surveys and to describing clinical features and outcomes. Moreover, no prospective studies on large series have been published to date, making therapeutic decisions difficult. Data regarding clinical and biological prognostic markers are limited, and it is therefore difficult to predict those in whom the disease will be more aggressive. This review will present recent data describing the epidemiology, clinical features, staging, and therapy of these lymphomas. EpidemiologyMZL account for between 5% and 17% of all non-Hodgkin lymphomas (NHL) in adults depending on the series. MALT lymphoma is the most frequent of the MZL subtypes, representing 50% to 70% of MZL and 7% to 8% of NHL. The splenic and the nodal MZL represent 20% and 10% of MZL, respectively, and account for less than 1% of NHL. Most of the cases occur in adults, with a median age of approximately 60 years, except for splenic MZL with villous lymphocytes (SLVL), occurring in adults at a median age of around 70 years. [4][5][6][7][8]

The spectrum of MALT lymphoma at different sites: biological and therapeutic relevance

“…145 Antibiotic therapy in gastric diffuse large B-cell lymphoma Although patients with gastric DLBCL can achieve tumor regression after anti-Helicobacter therapy, [82][83][84] additional evidence derived from large prospective studies is needed before routinely adopting this approach, and, at present, we recommend treating gastric largecell lymphomas according to the guidelines for localized DLBCL. 146 Antibiotic therapy as first-line treatment of these patients is not advised outside of clinical trials until evidence is derived from large prospective studies.…”

Nongastric Marginal-Zone B-Cell MALT Lymphoma: Prognostic Value of Disease Dissemination

“…Marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) are extranodal low-grade lymphomas that represent 8% of all non-Hodgkin's lymphomas (NHLs) [1][2][3]. MALT lymphomas are indolent disorders that typically affect patients of middle and advanced age, predominantly women [2][3][4][5][6].…”

“…MALT lymphomas are indolent disorders that typically affect patients of middle and advanced age, predominantly women [2][3][4][5][6].…”

Extranodal Marginal Zone Lymphoma of Ocular Adnexa: Outcomes following Radiation Therapy

“…Marginal zone B-cell lymphomas (MZL) are a group of lymphomas that originate from B-cell lymphocytes in the marginal zone of secondary lymphoid follicles [1]. MZL accounts for between 5 and 17% of all non-Hodgkin lymphomas (NHL) [2,3,4,5,6].…”