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T cell receptor gene rearrangements define a monoclonal T cell
proliferation in patients with T cell lymphocytosis and cytopenia
N Berliner, AD Duby, DC Linch, C Murre, T Quertermous, LJ Knott, T Azin, AC Newland, DL Lewis and MC Galvin
We have used probes from the T cell receptor beta and gamma chain loci to
investigate the clonality of T lymphocytes in eight patients with T cell
lymphocytosis and cytopenia (TCLC). This syndrome, which is strongly
associated with rheumatoid arthritis, is characterized by peripheral blood
and bone marrow lymphocytosis and neutropenia, red cell aplasia, or both.
By means of T cell monoclonal antibodies and flow cytometry, T lymphocytes
from patients with this syndrome have been shown to have characteristic
immunologic features. Investigators have disagreed as to whether the
syndrome represents a T cell malignancy or a more benign immunologic
disorder. DNA from five of five patients with symptomatic "classic" T cell
lymphocytosis with cytopenia demonstrated unique rearrangements of the T
cell receptor beta chain locus, whereas neither of two patients with
atypical features showed rearrangement. In addition, we found evidence for
gamma chain rearrangement in those DNAs with clonal beta chain
rearrangement. We thus postulate that the classic form of this syndrome is
associated with a monoclonal proliferation of T cells. Its potential
relationship to T cell chronic lymphocytic leukemia is discussed.
Volume 67,
Issue 4,
pp. 914-918,
04/01/1986
Copyright © 1986 by The American Society of Hematology
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