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Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of malignant lymphomas that share the propensity for malignant T lymphocytes that express cutaneous lymphocyte antigen (CLA) to infiltrate the skin. Mycosis fungoides (MF) is the most common variant of CTCL, representing 50 percent of all cases. Sézary syndrome (SS) is a leukemic variant of MF, affecting approximately 5 percent of patients with MF. MF and SS are the most common malignant proliferations of mature memory T lymphocytes of the helper phenotype (CD4+CD45RO+), 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. There are numerous therapeutic options available. However, no therapy has been definitively shown to improve survival. In early stages, the disease follows an indolent course and has favorable prognosis. In advanced stages, the prognosis is poor. 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. The goal of therapy for MF and SS is to induce long-term remissions without further compromising a patient’s immune system or quality of life.

Acronyms and Abbreviations

Acronyms and abbreviations used in this chapter include: CD, cluster of differentiation; CLA, cutaneous lymphocyte antigen; CTCL, cutaneous T-cell lymphoma; EBT, electron beam therapy; EORTC, European Organization for Research and Treatment of Cancer; Ig, immunoglobulin; MF, mycosis fungoides; NCCN, National Comprehensive Cancer Network; NK, natural killer; PUVA, psoralen ultraviolet A; SS, Sézary syndrome; Th2, T-helper type 2; TNMB, tumor, node, metastasis, blood; UV, ultraviolet light; WHO, World Health Organization.

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common malignant proliferations of mature memory T lymphocytes of the helper phenotype (CD4+CD45RO+).1 In 1806, Baron Jean-Louis Alibert described a patient who presented with skin patches that grew into plaques and mushroom-like tumors and first coined the term mycosis fungoides.2 In 1938, Sézary and Bouvrain described a syndrome of pruritus, generalized exfoliative erythroderma, and abnormal hyperconvoluted lymphoid cells in the blood.3 Today this condition is referred to as Sézary syndrome, a condition seen in a subset of patients with MF.

Prior to the 1970s, cutaneous lymphomas were believed to be cutaneous counterparts of the systemic lymphomas. In 1975, Lutzner and associates4 suggested the term cutaneous T-cell lymphoma (CTCL), recognizing that these cutaneous lymphomas have significant similarities of malignant cell morphology and phenotype and represent separate entities different from their systemic counterpart. This definition has helped to distinguish cutaneous lymphomas from systemic disease; however, it also led to ...

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