title: Cutaneous T-cell lymphoma (CTCL): Current practices in blood assessment and the utility of T-cell receptor (TCR)-Vβ chain restriction.
abstract: BACKGROUND
  Accurate quantification of malignant cells in the peripheral blood of patients with cutaneous T-cell lymphoma is important for early detection, prognosis, and monitoring disease burden.
  
  
  OBJECTIVE
  We sought to determine the spectrum of current clinical practices; critically evaluate elements of current International Society for Cutaneous Lymphomas (ISCL) B1 and B2 staging criteria; and assess the potential role of T-cell receptor-Vβ analysis by flow cytometry.
  
  
  METHODS
  We assessed current clinical practices by survey, and performed a retrospective analysis of 161 patients evaluated at Yale (2011-2014) to compare the sensitivity, specificity, positive predictive value, and negative predictive value of parameters for ISCL B2 staging.
  
  
  RESULTS
  There was heterogeneity in clinical practices among institutions. ISCL B1 criteria did not capture 5 Yale cohort cases with immunophenotypic abnormalities that later progressed. T-cell receptor-Vβ testing was more specific than polymerase chain reaction and aided diagnosis in detecting clonality, but was of limited benefit in quantification of tumor burden.
  
  
  CONCLUSIONS
  Because of limited follow-up involving a single center, further investigation will be necessary to conclude whether our proposed diagnostic algorithm is of general clinical benefit.
  
  
  CONCLUSIONS
  We propose further study of modified B1 criteria: CD4/CD8 ratio 5 or greater, %CD4(+) CD26(-) 20% or greater, or %CD4(+) CD7(-) 20% or greater, with evidence of clonality. T-cell receptor-Vβ testing should be considered in future diagnostic and staging algorithms.
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Juliet F
authors.initials: JF
authors.lastname: Gibson
authors.affiliation: Department of Dermatology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, New York.
authors.forename: Jing
authors.initials: J
authors.lastname: Huang
authors.affiliation: Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Kristina J
authors.initials: KJ
authors.lastname: Liu
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Kacie R
authors.initials: KR
authors.lastname: Carlson
authors.affiliation: Department of Hematology and Oncology, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Francine
authors.initials: F
authors.lastname: Foss
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Jaehyuk
authors.initials: J
authors.lastname: Choi
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, Connecticut.
authors.forename: Richard
authors.initials: R
authors.lastname: Edelson
authors.affiliation: Department of Pathology/ARUP Laboratories, University of Utah, Salt Lake City, Utah.
authors.forename: Jerry W
authors.initials: JW
authors.lastname: Hussong
authors.affiliation: Department of Pathology/ARUP Laboratories, University of Utah, Salt Lake City, Utah.
authors.forename: Ramsey
authors.initials: R
authors.lastname: Mohl
authors.affiliation: Department of Pathology/ARUP Laboratories, University of Utah, Salt Lake City, Utah.
authors.forename: Sally
authors.initials: S
authors.lastname: Hill
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, Connecticut. Electronic address: michael.girardi@yale.edu.
authors.forename: Michael
authors.initials: M
authors.lastname: Girardi
mesh_terms: D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D006403:Hematologic Tests; D006801:Humans; D038622:Internationality; D016410:Lymphoma, T-Cell, Cutaneous / Q000097:blood* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood / Q000473:pathology; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D016133:Polymerase Chain Reaction / Q000379:methods; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D011379:Prognosis; D035583:Rare Diseases; D011948:Receptors, Antigen, T-Cell / Q000276:immunology*; D012189:Retrospective Studies; D012751:Sezary Syndrome / Q000097:blood / Q000473:pathology; D012878:Skin Neoplasms / Q000097:blood* / Q000473:pathology; D012955:Societies, Medical / Q000592:standards
chemical_list: D011948:Receptors, Antigen, T-Cell
keywords: Sézary syndrome; T-cell receptor-Vβ; cutaneous T-cell lymphoma; flow cytometry; mycosis fungoides; peripheral blood analysis