Immunobiology and treatment of cutaneous T-cell lymphoma

ABSTRACT

Introduction

Primary cutaneous T cell lymphomas (CTCL) are a heterogenous group of non-Hodgkin lymphomas derived from skin-homing T cells. These include mycosis fungoides and its leukemic variant Sezary syndrome, as well as the CD30+ lymphoproliferative disorders.

Areas Covered

In this review, we provide a summary of the current literature on CTCL, with a focus on the immunopathogenesis and treatment of mycosis fungoides and Sezary syndrome.

Expert Opinion

Recent advances in immunology have provided new insights into the biology of malignant T cells. This in turn has led to the development of new therapies that modulate the immune system to facilitate tumor clearance or target specific aspects of tumor biology.

KEYWORDS:

• Cutaneous T cell lymphoma
• CTCL
• mycosis fungoides
• Sezary syndrome
• cutaneous oncology
• immunotherapy

Article highlights

• Cutaneous T cell lymphoma (CTCL) is a malignancy of skin-homing T cells that can range from skin-limited disease to leukemic involvement (Sezary syndrome)

• CTCL results in a number of immune derangements that subvert the endogenous anti-tumor response

• Early-stage CTCL is often managed with topical therapies whereas management of advanced CTCL often includes systemic therapies that specifically target aspects of tumor biology and augment anti-tumor immune responses

• Research is ongoing to identify additional therapies and identify potential biomarkers of treatment response

Declaration of interest

A Rook is a lecturer for Mallinckrodt and a consultant for TLR Bio. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.