Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions

Abstract

Both mycosis fungoides (MF) and Sézary syndrome (SS) have a chronic, relapsing course, with patients frequently undergoing multiple, consecutive therapies. Treatment is aimed at the clearance of skin disease, the minimization of recurrence, the prevention of disease progression, and the preservation of quality of life. Other important considerations are symptom severity, including pruritus and patient age/comorbidities. In general, for limited patch and plaque disease, patients have excellent prognosis on ≥1 topical formulations, including topical corticosteroids and nitrogen mustard, with widespread patch/plaque disease often requiring phototherapy. In refractory early stage MF, transformed MF, and folliculotropic MF, a combination of skin-directed therapy plus low-dose immunomodulators (eg, interferon or bexarotene) may be effective. Patients with advanced and erythrodermic MF/SS can have profound immunosuppression, with treatments targeting tumor cells aimed for immune reconstitution. Biologic agents or targeted therapies either alone or in combination--including immunomodulators and histone-deacetylase inhibitors--are tried first, with more immunosuppressive therapies, such as alemtuzumab or chemotherapy, being generally reserved for refractory or rapidly progressive disease or extensive lymph node and metastatic involvement. Recently, an increased understanding of the pathogenesis of MF and SS with identification of important molecular markers has led to the development of new targeted therapies that are currently being explored in clinical trials in advanced MF and SS.

Keywords: BSA; CR; CRR; CTCL; ECP; EORTC; European Organization of Research and Treatment of Cancer; HDACi; IFNα; ISCL; International Society for Cutaneous Lymphoma; MF; NBUVB; NCCN; NK; NM; NMSC; National Comprehensive Cancer Network; ORR; PUVA; RAR; RXR; SS; Sézary syndrome; TNMB; TSEBT; USCLC; UVB; United States Cutaneous Lymphoma Consortium; body surface area; complete response; complete response rate; cutaneous T-cell lymphoma; extracorporeal photopheresis; histone deacetylase inhibitor; immunomodulators; interferon-alfa; mSWAT; modified severity-weighted assessment tool; mycosis fungoides; narrowband ultraviolet B light; natural killer; nitrogen mustard; nonmelanoma skin cancer; overall response rate; phototherapy; prognosis; psoralen plus ultraviolet A light phototherapy; retinoic acid receptor; retinoid X receptor; skin-directed treatment; staging; systemic treatment; targeted therapies; topical nitrogen mustard; topical retinoids/rexinoids; topical corticosteroids; total skin electron beam therapy; tumor, node, metastasis, blood; ultraviolet B light.