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Review
. 2021 Mar;84(3):587-595.
doi: 10.1016/j.jaad.2020.12.027. Epub 2020 Dec 22.

The immunopathogenesis and immunotherapy of cutaneous T cell lymphoma: Pathways and targets for immune restoration and tumor eradication

Joseph S Durgin  1 , David M Weiner  1 , Maria Wysocka  1 , Alain H Rook  2
Affiliations
Review

The immunopathogenesis and immunotherapy of cutaneous T cell lymphoma: Pathways and targets for immune restoration and tumor eradication

Joseph S Durgin et al. J Am Acad Dermatol. 2021 Mar.

Abstract

Cutaneous T cell lymphomas (CTCLs) are malignancies of skin-trafficking T cells. Patients with advanced CTCL manifest immune dysfunction that predisposes to infection and suppresses the antitumor immune response. Therapies that stimulate immunity have produced superior progression-free survival compared with conventional chemotherapy, reinforcing the importance of addressing the immune deficient state in the care of patients with CTCL. Recent research has better defined the pathogenesis of these immune deficits, explaining the mechanisms of disease progression and revealing potential therapeutic targets. The features of the malignant cell in mycosis fungoides and Sézary syndrome are now significantly better understood, including the T helper 2 cell phenotype, regulatory T cell cytokine production, immune checkpoint molecule expression, chemokine receptors, and interactions with the microenvironment. The updated model of CTCL immunopathogenesis provides understanding into clinical progression and therapeutic response.

Keywords: CTCL; Sézary syndrome; cutaneous T cell lymphoma; dermatologic oncology; drug response; immune deficiency; immunopathogenesis; immunotherapy; mycosis fungoides.

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Conflict of interest statement

Conflicts of Interest: MW and AHR are inventors on a pending patent for the use of resiquimod for cutaneous T cell lymphoma.

Figures

Figure 1.
Figure 1.
“Sézary syndrome. Malignant cells dysregulate the host immune response through surface and secreted factors.”
See this image and copyright information in PMC

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