The clonal nature of circulating Sezary cells [published erratum appears in Blood 1996 Jun 1;87(11):4923]

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.