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Marginal zone B-cell lymphomas of different sites share similar cytogenetic
and morphologic features [see comments]
J Dierlamm, S Pittaluga, I Wlodarska, M Stul, J Thomas, M Boogaerts, L Michaux, A Driessen, C Mecucci and JJ Cassiman
Department of Pathology, University of Leuven, Belgium.
Clinical, histologic, cytogenetic, and molecular genetic data of 31
patients with extranodal, nodal, and splenic marginal zone B-cell lymphoma
(MZBCL) are presented. Despite these variable clinical manifestations, a
similar spectrum of morphologic features as well as distinctive
immunophenotypic findings were noted. In all cases, a monotypic B-cell
proliferation consistently negative for CD5, CD10, and CD23 was found
expanding the marginal zone of the B follicle with and without colonization
of the follicle centers. Clonal chromosomal abnormalities were detected in
23 of the 31 patients. Recurrent aberrations included whole or partial
trisomy 3 (18 cases), trisomy 18 (9 cases), and structural rearrangements
of chromosome 1 with breakpoints in 1q21 (9 cases) or 1p34 (6 cases), all
of which were seen in extranodal, nodal, as well as splenic MZBCL.
Abnormalities of the additional chromosome 3, such as
+del(3)(p13),+i(3)(q10), or structural changes involving the distal part of
the long arm, were evident in 9 of the 18 cases. All recurrent
abnormalities were found in MZBCL more frequently than in other histologic
entities of B-cell non-Hodgkin's lymphoma (B-NHL). None of the known
lymphoma-associated chromosomal changes or rearrangements of the BCL1,
BCL2, BCL3, BCL6, and CMYC genes were detected. We conclude that MZBCL
represent a distinct entity of B- NHL with characteristic morphologic and
immunophenotypic features and particular chromosomal abnormalities, and
that a close histogenetic relationship between extranodal, nodal, and
splenic MZBCL is likely, although the clinical presentation may vary.
Volume 87,
Issue 1,
pp. 299-307,
01/01/1996
Copyright © 1996 by The American Society of Hematology
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