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INTRODUCTION

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SUMMARY

Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of malignant lymphomas that share the propensity for malignant T lymphocytes expressing cutaneous lymphocyte antigen to infiltrate the skin. Mycosis fungoides (MF) is the most common variant of CTCL, representing approximately 50 percent of all cases. Sézary syndrome (SS) is a leukemic variant of MF, affecting approximately 5 percent of patients with MF.1 MF and SS are the most common malignant proliferations of mature memory T lymphocytes of the helper phenotype (CD4+CD45RO+),2 which renders patients immunocompromised even at the earliest stages of the disease. Advanced stages are associated with severe immune suppression. Diagnosis is established by skin biopsy, followed by staging work up which includes radiologic imaging and pathologic evaluation of the lymph nodes, internal organs, blood, and marrow, as appropriate, according to presenting manifestations of the disease.

MF is divided into early and advanced stages for therapeutic and prognostic reasons. In early stages, the disease follows an indolent course and has a favorable prognosis. In advanced stages, the prognosis is poor. There are numerous therapeutic options. No treatment has been definitively proven to improve survival, but newer studies suggest that survival is longer than historically documented.3,4 Multiagent chemotherapy is not a useful option because it is inferior to immune modulating and biologic therapies.5 Considering the overall protracted course of the disease, its indolent character, immunocompromised status of the patients, and absence of definitive therapy, aggressive multiagent chemotherapy contributing to immunosuppression should be reserved for end-stage palliation or as a bridge to stem cell transplantation with the goal of definitive cure.6,7,8 There are several FDA-approved single agents for therapy of MF, which are safe and effective against the disease and may be used in the therapeutic ladder prior to multi-agent regimens.9,10,11 Several single agents and combinations of new and older agents are in clinical trials now to test for efficacy in MF and SS. Because no single therapy is considered to be the standard of care for MF and SS, clinical trials remain a viable option for most patients. The goal of therapy is to induce long-term remissions without compromising patients’ immunity and improvement of the quality of life.

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Acronyms and Abbreviations

ALCL, anaplastic large cell lymphoma; ALK-1, anaplastic lymphoma kinase-1; ATRA all-trans retinoic acid; BCNU, bis-chloroethyl-nitrosourea or carmustine; CLA, cutaneous lymphocyte antigen; CT, computed tomography; CTCL, cutaneous T-cell lymphoma; DD, denileukin diftitox; ECP, extracorporeal photopheresis; EORTC, European Organisation for Research and Treatment of Cancer; HDACi, histone deacetylase inhibitor; HTLV-1, human T-lymphotropic virus type 1; Ig, immunoglobulin; IL, interleukin; LEBT, localized electron beam therapy; LyP, lymphomatoid papulosis; MF, mycosis fungoides; MMAE, monomethyl auristatin E; NBUVB, narrow band UVB; NCCN, National Comprehensive Cancer Network; NK, natural killer; PCALCL, primary cutaneous anaplastic large cell lymphoma; PCR, polymerase chain reaction; PET, positron emission tomography; PUVA, psoralen with UVA; RXR, retinoid X receptor; SDT, skin-directed therapy; ...

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