Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

Thorbjørn Frej Krejsgaard; Niels Ødum; Carsten Geisler; M.A. Wasik; Anders Woetmann

doi:10.1038/leu.2011.237

Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

Thorbjørn Frej Krejsgaard, Niels Ødum, Carsten Geisler, M.A. Wasik, Anders Woetmann

72 Citations (Scopus)

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.

Original language	English
Journal	Leukemia
Volume	26
Pages (from-to)	424-432
Number of pages	9
ISSN	0887-6924
DOIs	https://doi.org/10.1038/leu.2011.237
Publication status	Published - Mar 2012

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title = "Regulatory T cells and immunodeficiency in mycosis fungoides and S{\'e}zary 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 S{\'e}zary 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.",

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T1 - Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

AU - Krejsgaard, Thorbjørn Frej

AU - Ødum, Niels

AU - Geisler, Carsten

AU - Wasik, M.A.

AU - Woetmann, Anders

PY - 2012/3

Y1 - 2012/3

N2 - 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.

AB - 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.

U2 - 10.1038/leu.2011.237

DO - 10.1038/leu.2011.237

M3 - Journal article

C2 - 21904385

SN - 0887-6924

VL - 26

SP - 424

EP - 432

JO - Leukemia

JF - Leukemia

ER -

Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

Abstract

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