title: Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome.
abstract: Cutaneous T-cell lymphoma (CTCL) is the term for diseases characterized by primary accumulation of malignant T cells in the skin. Patients with the two predominant clinical forms of CTCL called mycosis fungoides (MF) and Sézary syndrome (SS) characteristically develop severe immunodeficiency during disease progression and consequently patients with advanced disease frequently die of infections and not from the tumor burden. For decades, it has been suspected that the malignant T cells actively drive the evolving immunodeficiency to avoid antitumor immunity, yet, the underlying mechanisms remain unclear. The identification of a subset of highly immunosuppressive regulatory T cells (Tregs) triggered a variety of studies investigating if MF and SS are malignant proliferations of Tregs but seemingly discordant findings have been reported. Here, we review the literature to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency.
authors: Department of International Health, Immunology and Microbiology, University of Copenhagen, Copenhagen, Denmark. Thorkr@sund.ku.dk
authors: T
authors: T
authors: Krejsgaard
authors: N
authors: N
authors: Odum
authors: C
authors: C
authors: Geisler
authors: M A
authors: MA
authors: Wasik
authors: A
authors: A
authors: Woetmann
mesh_terms: D000818:Animals; D015704:CD4 Antigens / Q000378:metabolism; D051858:Forkhead Transcription Factors / Q000378:metabolism; D006801:Humans; D007111:Immunity, Cellular; D053645:Interleukin-2 Receptor alpha Subunit / Q000378:metabolism; D009182:Mycosis Fungoides / Q000276:immunology* / Q000378:metabolism; D012751:Sezary Syndrome / Q000276:immunology* / Q000378:metabolism; D050378:T-Lymphocytes, Regulatory / Q000276:immunology* / Q000378:metabolism
chemical_list: D015704:CD4 Antigens; C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; D053645:Interleukin-2 Receptor alpha Subunit