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Nonfollicular small B-cell lymphomas: a heterogeneous group of patients
with distinct clinical features and outcome
F Berger, P Felman, A Sonet, G Salles, Y Bastion, PA Bryon and B Coiffier
Service d'Anatomie Pathologique, Hopital Edouard-Herriot, Lyon, France.
Two hundred sixteen patients with a nonfollicular small cell lymphoma
followed up in our department over a 5-year period have been reviewed to
define the clinical behavior and survival of patients with each histologic
subtype. The respective frequencies of major subtypes were: small
lymphocytic/lymphoplasmacytoid lymphoma (immunocytoma, SL/LPL), 28%; large
cell-rich immunocytoma (LCRI), 7%; mantle cell lymphoma (MCL), 24%;
mucosa-associated lymphoid tissue-lymphoma (MALT-L), 20%; other rare
subtypes, 6%; and nonclassified or nonreviewed, 14%. The SL/LPL patients
and the MALT-L patients had a relatively indolent disease, usually
disseminated for SL/LPL and usually localized for MALT- L. Both subtypes
have a long time to treatment failure (TTF; median, 48 and 58 months,
respectively) and long survival (median, 118 and 98 months, respectively).
The LCRI patients or the MCL patients had more aggressive clinical or
biologic features and experienced shorter TTF (median, 26 and 14 months,
respectively) and shorter survival (median, 55 and 52 months,
respectively). None of these histologic subtypes was associated with a
significant cure rate. MALT-L patients did relapse regardless of the
initial localization or treatment and at a similar rate to the SL/LPL
patients. Factors associated with a worse outcome in nonfollicular small
cell lymphoma patients are identical to those described in other lymphoma
subtypes: advanced clinical stage, poor performance status, high tumor
bulk, and high lactic dehydrogenase or beta 2microglobulin levels. For
patients with disseminated disease, standard chemotherapy regimens did not
allow a long TTF; therefore, new therapeutic strategies must be developed.
Volume 83,
Issue 10,
pp. 2829-2835,
05/15/1994
Copyright © 1994 by The American Society of Hematology
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