title: CD164 and FCRL3 are highly expressed on CD4+CD26- T cells in Sézary syndrome patients.
abstract: Sézary syndrome (SS) cells express cell surface molecules also found on normal activated CD4 T cells. In an effort to find a more specific surface marker for malignant SS cells, a microarray analysis of gene expression was performed. Results showed significantly increased levels of mRNA for CD164, a sialomucin found on human CD34+ hematopoietic stem cells, and FCRL3, a molecule present on a subset of human natural T regulatory cells. Both markers were increased in CD4 T cells from SS patients compared with healthy donors (HD). Flow cytometry studies confirmed the increased expression of CD164 and FCRL3 primarily on CD4+CD26- T cells of SS patients. Importantly, a statistically significant correlation was found between an elevated percentage of CD4+CD164+ T cells and an elevated percentage of CD4+CD26- T cells in all tested SS patients but not in patients with mycosis fungoides and atopic dermatitis or HD. FCRL3 expression was significantly increased only in patients with high tumor burden. CD4+CD164+ cells displayed cerebriform morphology and their loss correlated with clinical improvement in treated patients. Our results suggest that CD164 can serve as a marker for diagnosis and for monitoring progression of cutaneous T-cell lymphoma (CTCL)/SS and that FCRL3 expression correlates with a high circulating tumor burden.
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA. Electronic address: mwysocka@mail.med.upenn.edu.
authors.forename: Maria
authors.initials: M
authors.lastname: Wysocka
authors.affiliation: The Wistar Institute, Philadelphia, Pennsylvania, USA.
authors.forename: Andrew V
authors.initials: AV
authors.lastname: Kossenkov
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Bernice M
authors.initials: BM
authors.lastname: Benoit
authors.affiliation: Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Andrea B
authors.initials: AB
authors.lastname: Troxel
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Elisha
authors.initials: E
authors.lastname: Singer
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Andras
authors.initials: A
authors.lastname: Schaffer
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Brian
authors.initials: B
authors.lastname: Kim
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Tzvete
authors.initials: T
authors.lastname: Dentchev
authors.affiliation: Cancer Biology Research Center, Sanford Research/USD, Sioux Falls, South Dakota, USA.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Nagata
authors.affiliation: Cancer Biology Research Center, Sanford Research/USD, Sioux Falls, South Dakota, USA.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Ise
authors.affiliation: The Wistar Institute, Philadelphia, Pennsylvania, USA.
authors.forename: Louise C
authors.initials: LC
authors.lastname: Showe
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology* / Q000378:metabolism; D048430:Cell Shape / Q000276:immunology; D018819:Dipeptidyl Peptidase 4 / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D018450:Disease Progression; D051930:Endolyn / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D005434:Flow Cytometry; D006801:Humans; D008562:Membrane Glycoproteins / Q000235:genetics; D008840:Microfilament Proteins / Q000235:genetics; D009360:Neoplastic Cells, Circulating / Q000276:immunology* / Q000378:metabolism; D011971:Receptors, Immunologic / Q000276:immunology* / Q000378:metabolism; D012751:Sezary Syndrome / Q000276:immunology*; D059467:Transcriptome
chemical_list: D015415:Biomarkers; D051930:Endolyn; C457775:FCRL3 protein, human; D008562:Membrane Glycoproteins; D008840:Microfilament Proteins; D011971:Receptors, Immunologic; C026742:plastin; C042807:DPP4 protein, human; D018819:Dipeptidyl Peptidase 4