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Blood, Vol. 95 No. 6 (March 15), 2000:
pp. 1950-1956
Non-MALT marginal zone B-cell lymphomas: a description of clinical
presentation and outcome in 124 patients
Françoise Berger,
Pascale Felman,
Catherine Thieblemont,
Thierry Pradier,
Lucille Baseggio,
Paul-Andre Bryon,
Gilles Salles,
Evelyne Callet-Bauchu, and
Bertrand Coiffier
From the Service d'Anatomie Pathologique and Laboratoire
d'Hèmatologie, Hùpital Edouard-Herriot, Lyon, France;
Laboratoire d'Hèmatologie, Service d'Hèmatologie, and
Service de Chirurgie Gènèrale, Centre Hospitalier Lyon-Sud,
Pierre Bènite, France.
Marginal zone B-cell lymphoma (MZL) is a recently individualized
lymphoma that encompasses mucosa-associated lymphoid tissue (MALT)
lymphoma, splenic lymphoma with or without villous lymphocytes, and
nodal lymphoma with or without monocytoid B-cells. If the clinical
description and outcome of MALT lymphoma is well known, this is not the
case for the other subtypes. We reviewed 124 patients presenting
non-MALT MZL treated in our department to describe the morphologic and
clinical presentation and the outcome of these lymphomas. Four clinical
subtypes were observed: splenic, 59 patients; nodal, 37 patients;
disseminated (splenic and nodal), 20 patients; and leukemic (not
splenic nor nodal), 8 patients. These lymphomas were usually CD5-,
CD10-, CD23-, and CD43-, but the detection of one or, rarely, two of
these antigens may be observed. Bone marrow and blood infiltrations
were frequent, except in the nodal subtype, but these locations were
not associated with a poorer outcome. Splenic and leukemic subtypes
were associated with a median time to progression (TTP) longer than 5 years, even in the absence of treatment or of complete response to
therapy. Nodal and disseminated subtypes were associated with a median
TTP of 1 year. However, in all these subtypes, survival was good with a
median survival of 9 years, allowing these lymphomas to be classified as indolent. Because of the retrospective nature of this analysis, no
conclusion may be drawn on the therapeutic aspects, but conservative treatments seem recommended for leukemic and splenic subtypes.
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