Cutaneous T-cell lymphoma

Cutaneous T-cell lymphomas (CTCL) diagnosis is based on a combination of clinical, histologic, immunophenotypic, and genetic data, and usually requires specialist expertise.

Establishing a diagnosis can be challenging, as the condition can take many different forms in the skin: flat patches, raised plaques, large tumors, and/or marked erythroderma (intense and widespread reddening of the skin).

Early-stage disease is usually managed with skin-directed therapy (topical treatments, phototherapy, and localized radiation therapy). If skin disease progresses, or the patient presents with advanced disease, systemic therapies are often necessary (chemotherapy, biologic or immunologic therapy, photopheresis).

The choice of skin-directed therapy or systemic treatment is dependent on both doctor and patient preference; no one treatment option has been shown to be superior to another. Clinical trials may be considered in early or advanced disease.

Patients diagnosed with early-stage disease have a higher rate of survival. Treatment may induce remission, but patients are expected to relapse after a variable interval as complete cure is rare.

CTCL make up a heterogeneous group of uncommon disorders characterized by clonal accumulation of T lymphocytes primarily or exclusively in the skin. Mycosis fungoides (MF) and its leukemic variant, Sézary syndrome (SS), are the most common subtypes.[1]Olsen EA, Whittaker S, Kim YH, et al. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol. 2011 Jun 20;29(18):2598-607. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3422534 http://www.ncbi.nlm.nih.gov/pubmed/21576639?tool=bestpractice.com MF frequently behaves as an indolent lymphoma with good prognosis for early-stage disease, while SS is considered a more aggressive form of the disease, associated with shortened survival.

This topic addresses the diagnosis and treatment of MF and SS.