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Mutation Analysis of the Rearranged Immunoglobulin Heavy Chain
Genes of Marginal Zone Cell Lymphomas Indicates an Origin From
Different Marginal Zone B Lymphocyte Subsets
A. Tierens,
J. Delabie,
S. Pittaluga,
A. Driessen, and
C. DeWolf-Peeters
From the Laboratory of Hematology, University Hospitals of Leuven;
and the Department of Pathology II, University Hospitals of Leuven,
Leuven, Belgium.
Marginal zone cell lymphoma is a recently described entity among the
non-Hodgkin's lymphomas. It likely originates from the marginal zone B
cells in the spleen and equivalent cells in the lymph node and
extranodal tissues. Recent evidence indicates that marginal zone B
cells are functionally heterogeneous and may differ with respect to the
pattern of somatic hypermutation in their Ig variable genes. To test
whether marginal zone lymphomas may originate from different subsets of
marginal zone B cells, we performed a sequence and mutation analysis of
the rearranged Ig heavy chain (IgH) variable genes
(VH) of a series of 14 cases of
marginal zone lymphoma, occurring in the spleen (4), the lymph node
(4), the stomach (2), the orbit (2), the tongue (1), and the skin (1).
Our data show that marginal zone cell lymphomas preferentially
rearrange the VH4, VH3,
and VH1 family genes, without preference for
any particular VH gene. Somatic mutations are
present in 13 cases; one case of marginal zone cell lymphoma of the
skin showed a germline configuration of the rearranged
VH gene. Mutation analysis shows evidence of
antigen selection in three cases of marginal zone cell lymphoma, one of
the spleen, stomach, and orbit, respectively. No evidence of antigen
selection was present in the other cases. These data indicate that
marginal zone cell lymphomas may arise from different subsets of
marginal zone B cells. In addition, lymphomagenesis may not be
triggered by antigen in all cases of marginal zone cell lymphoma.
Blood, Vol. 91 No. 7 (April 1), 1998:
pp. 2381-2386
© 1998 by The American Society of Hematology.

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