title: Molecular detection of circulating Sezary cells in patients with mycosis fungoides: could it predict future development of secondary Sezary syndrome? A single-institution experience.
abstract: While the majority of patients with early-stage mycosis fungoides (MF) have an excellent prognosis, a few cases progress to secondary Sezary syndrome (sSS), which carries a dismal clinical outcome. We retrospectively analyzed 135 cases of MF/SS and correlated molecular detection of T-cell clones in the skin and blood with other clinicopathologic findings. When stratified by the diagnoses, patients with MF demonstrated a 26.5% (31/117) positive rate for a blood T-cell clone, of which 50% (10/20) had an identical T-cell clone in the skin. Follow-up evaluation showed conversion into sSS or leukemic phase in 50% (5/10) of cases with a positive blood T-cell clone (estimated mean interval 41.8 months) in comparison to no cases in the group without a clone (0/31). Interestingly, 4/5 cases of sSS had an identical T-cell clone in the skin, while the remaining case did not have the test performed on skin for clonal comparison. Kaplan-Meier survival analysis demonstrated a poor clinical outcome in the group with a blood T-cell clone, in comparison with the group without, in overall survival (p < 0.0001) and progression-free survival (p < 0.0001; HR = 22.6). These findings suggest that molecular detection of a blood T-cell clone may have a role in predicting sSS. Due to amplification of non-neoplastic T-cell expansion in a significant number of cases, comparison of blood T-cell clones with skin may have confirmatory value.
authors: Department of Pathology, Duke University Medical Center, Durham, NC, USA.
authors: Charles Blake
authors: CB
authors: Hutchinson
authors: Maggie
authors: M
authors: Stoecker
authors: Frances F
authors: FF
authors: Wang
authors: John
authors: J
authors: Papalas
authors: Siby
authors: S
authors: Sebastian
authors: James
authors: J
authors: Burchette
authors: Michael
authors: M
authors: Datto
authors: Endi
authors: E
authors: Wang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002999:Clone Cells / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000473:pathology*; D016609:Neoplasms, Second Primary; D009360:Neoplastic Cells, Circulating / Q000473:pathology*; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012751:Sezary Syndrome / Q000473:pathology*; D016019:Survival Analysis; D055815:Young Adult