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Cutaneous T cell Lymphoma: an Update on Pathogenesis and Systemic Therapy

  • T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)
  • Published:
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Abstract

Mycosis fungoides (MF) and its leukemic variant, Sézary syndrome (SS), are malignancies of skin-homing T cells that comprise the majority of cutaneous T cell lymphomas (CTCL). Treatment of CTCL is limited and can be approached by skin-directed therapy or systemic therapy. Recent investigations into the pathogenesis of MF and SS have broadened the therapeutic targets; here, we review emerging concepts in the pathogenesis of MF and SS as well as novel and traditional systemic therapies for MF and SS. These include histone deacetylase inhibitors (vorinostat, romidepsin, panobinostat, and belinostat), monoclonal antibodies (alemtuzumab, brentuximab vedotin, and mogamulizumab) and single-agent cytotoxic chemotherapeutic agents (e.g., pralatrexate, doxorubicin, bendamustine, and forodesine), as well as multi-agent chemotherapy regimens.

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Abbreviations

CTCL:

Cutaneous T cell lymphoma

L-CTCL:

Leukemic CTCL

MF:

Mycosis fungoides

TCM :

Central memory T cell

TEM :

Effector memory T cell

HDAC:

Histone deacetylase

DAC:

Deacetylase

FDA:

Food and Drug Administration

PTCL:

Peripheral T cell lymphoma

ORR:

Overall response rate

GI:

Gastrointestinal

IV:

Intravenous

CR:

Complete response

PR:

Partial response

CLL:

Chronic lymphocytic leukemia

SC:

Subcutaneous

ALCL:

Anaplastic large cell lymphoma

PDX:

Pralatrexate

MTX:

Methotrexate

IFN:

Interferon

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Authors and Affiliations

  1. Departments of Dermatology and Pathology, Penn State Hershey Medical Center, 500 University Drive, Hershey, PA, 17033, USA

    Catherine G. Chung

  2. Rochester Skin Lymphoma Medical Group, 6800 Pittsford Palmyra Road, Fairport, NY, 14450, USA

    Brian Poligone

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Catherine G. Chung and Brian Poligone each declare no potential conflicts of interest.

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This article is part of the Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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Chung, C.G., Poligone, B. Cutaneous T cell Lymphoma: an Update on Pathogenesis and Systemic Therapy. Curr Hematol Malig Rep 10, 468–476 (2015). https://doi.org/10.1007/s11899-015-0293-y

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