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Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome

Leukemia volume 26, pages 424–432 (2012)Cite this article

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.

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Acknowledgements

This study was supported in part by research funding from the Carlsberg Foundation (Carlsbergfondet), the Danish Research Councils, the Danish Cancer Society, the Lundbeck Foundation, the Novo Nordic Foundation, Fabrikant Vilhelm Pedersen og Hustrus Mindelegat, the Neye Foundation, the University of Copenhagen and the National Cancer Institute Grant (CA89194) (MAW).

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Authors and Affiliations

  1. Department of International Health, Immunology and Microbiology, University of Copenhagen, Copenhagen, Denmark

    T Krejsgaard, N Odum, C Geisler & A Woetmann

  2. Department of Biology, University of Copenhagen, Copenhagen, Denmark

    T Krejsgaard, N Odum & A Woetmann

  3. Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA

    M A Wasik

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  1. T Krejsgaard
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  2. N Odum
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  3. C Geisler
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Correspondence to T Krejsgaard.

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Krejsgaard, T., Odum, N., Geisler, C. et al. Regulatory T cells and immunodeficiency in mycosis fungoides and Sézary syndrome. Leukemia 26, 424–432 (2012). https://doi.org/10.1038/leu.2011.237

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  • DOI: https://doi.org/10.1038/leu.2011.237

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