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The clonal nature of circulating Sezary cells [published erratum appears in
Blood 1996 Jun 1;87(11):4923]
JM Weinberg, C Jaworsky, BM Benoit, B Telegan, AH Rook and SR Lessin
Department of Dermatology, University of Pennsylvania, Philadelphia 19104,
USA.
To determine if circulating Sezary cells can be classified as reactive or
neoplastic based on the ability to detect the presence or absence of clonal
T-cell receptor beta chain (TCR-beta) gene rearrangements by Southern blot
analysis, we evaluated the peripheral blood of 25 patients: 11 patients
with Sezary syndrome (SS), 11 with benign inflammatory dermatoses (BID),
and three normal controls. Three of 11 patients with SS, with Sezary counts
ranging from 14% to 52%, did not demonstrate any clonal TCR-beta gene
rearrangements in the peripheral blood, despite a TCR-beta rearrangement by
Southern blot analysis in the skin. Ten of 11 BID patients and all normal
controls showed no evidence of a TCR-beta gene rearrangement in the
peripheral blood. However, one patient with psoriasis demonstrated a
TCR-beta gene rearrangement in the peripheral blood. The TCR-beta gene
rearrangement detected in this patient, confirmed with polymerase chain
reaction (PCR) amplification of the TCR-gamma gene rearrangement, did not
correlate with the presence of circulating Sezary cells or the increased
risk of neoplasia. Our results indicate that circulating Sezary cells may
be monoclonal (neoplastic) or polyclonal (reactive), as defined by TCR gene
rearrangement studies. Circulating Sezary cells in SS may be reactive in
nature and not accurately reflect the actual tumor burden in the peripheral
blood. The presence of circulating Sezary cells or the presence of a clone
of cells defined by TCR-beta gene rearrangement in the peripheral blood is
not limited to neoplastic disease processes.
Volume 86,
Issue 11,
pp. 4257-4262,
12/01/1995
Copyright © 1995 by The American Society of Hematology
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