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Splenic lymphoma with villous lymphocytes involves B cells with extensively
mutated Ig heavy chain variable region genes
D Zhu, DG Oscier and FK Stevenson
Molecular Immunology Group, Tenovus Laboratory, Southampton University
Hospitals, UK.
Splenic lymphoma with villous lymphocytes (SLVL) is a recently defined
subgroup of chronic B-cell lymphoproliferative diseases. The characteristic
morphology of the tumor cells, together with phenotypic and cytogenetic
findings, indicate that it is a distinct entity, but the nature of the cell
or origin and its relationship to other low- grade lymphomas is unclear.
For B-cell tumors, analysis of the variable region heavy chain (VH) genes
used to encode the clonal Ig has shown marked differences between
histologic categories, both in gene usage and extent of somatic mutation.
An investigation of VH genes used in five typical cases of SLVL has shown
somatic hypermutation from germline sequences in all cases, indicating that
the cell of origin has been exposed to the hypermutation mechanism.
However, no clonal heterogeneity was detectable, demonstrating that the
tumor cell does not accumulate further mutations. These characteristics are
similar to those found in mature postfollicular B cells, such as plasma
cells. The distribution of mutations leading to replacement amino acids
differed among the cases, with three of five cases showing clear evidence
for antigen selection.
Volume 85,
Issue 6,
pp. 1603-1607,
03/15/1995
Copyright © 1995 by The American Society of Hematology
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