Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients

Marginal zone B-cell lymphoma (MZL) is a recently individualized lymphoma t hat encompasses mucosa-associated lymphoid tissue (MALT) lymphoma, splenic lymphoma with or without villous lymphocytes, and nodal lymphoma with or wi thout monocytoid B-cells, If the clinical description and outcome of MALT l ymphoma is well known, this is not the case for the other subtypes, We revi ewed 124 patients presenting non-MALT MZL treated in our department to desc ribe the morphologic and clinical presentation and the outcome of these lym phomas. Four clinical subtypes were observed: splenic, 59 patients; nodal, 37 patients; disseminated (splenic and nodal), 20 patients; and leukemic (n ot splenic nor nodal), 8 patients. These lymphomas were usually CD5-, CD10- , CD23-, and CD43-, but the detection of one or, rarely, two of these antig ens may be observed. Bone marrow and blood infiltrations were frequent, exc ept in the nodal subtype, but these locations were not associated with a po orer outcome. Splenic and leukemic subtypes were associated with a median t ime to progression (TTP) longer than 5 years, even in the absence of treatm ent or of complete response to therapy. Nodal and disseminated subtypes wer e associated with a median TTP of 1 year. However, in all these subtypes, s urvival was good with a median survival of 9 years, allowing these lymphoma s to be classified as indolent. Because of the retrospective nature of this analysis, no conclusion may be drawn on the therapeutic aspects, but conse rvative treatments seem recommended for leukemic and splenic subtypes, (C) 2000 by The American Society of Hematology.