Current Treatment Options for Cutaneous T-cell Lymphoma
Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas characterized by their surface markers and biological behavior. Mycosis fungoides (MF) and its leukemic variant Sézary syndrome (SS) are the most frequently encountered CTCLs resulting from a progressive clonal expansion of CD4+CD45RO+CLA+CCR+ helper/memory T cells. The malignant clones may have loss of common T-cell markers (CD7 and/or CD26).¹ Progression of MF/SS is accompanied by clonal dominance,² secretion of Th2 cytokines,³ impaired immune responses, and cell growth advantage.^1,4 The malignant T cells exhibit abnormal apoptotic mechanisms, such as loss of Fas or expression of Bc^l-2, that result in loss of activation-induced cell death, prolonged life span, and accumulation. These cells typically become resistant to treatment over time.⁵

The choice of therapy should be based on the stage of disease. Patients with early MF have disease limited to the skin (T1–2N0–1M0; IA–IIA) and can be put into remission with topical agents (topical steroids, retinoids, phototherapy or mustargen). More advanced or late stages (T1–4N0 –3M0–1; IIB–IVB) of MF or SS require both systemic and skin-directed therapies. Patients who have extensive refractory skin involvement, blood involvement, tumors, or nodal disease require systemic therapy. Refractory or extensive skin involvement including SS responds to systemic biologic response modifiers (retinoids, bexarotene, interferon, and denileukin diftitox), skin radiation and photopheresis. Single- or multi-agent chemotherapies are used for patients with large numbers of transformed tumors or nodal/visceral disease. In general, a combination of either sequential or concomitant therapies gives a higher rate of response, but advanced patients often relapse and curative therapy is elusive for most.¹

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