Cutaneous T-cell lymphoma

Diagnosis is based on clinical findings, skin biopsy, and laboratory blood tests, 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 tumours, and/or marked erythroderma (intense and widespread reddening of the skin).

Early-stage disease is usually managed with skin-directed therapy (topical medications, phototherapy, and localised radiotherapy). If skin disease progresses, or the patient presents with advanced disease, systemic therapies are often necessary (chemotherapy, biological or immunological therapy, photopheresis).

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

Prognosis is closely related to the stage at diagnosis. Survival rates in early disease are excellent. However, although treatment may induce remission, patients are expected to relapse after a variable interval and are unlikely to be cured.

Cutaneous T-cell lymphomas (CTCL) make up a heterogeneous group of uncommon disorders characterised by clonal accumulation of T lymphocytes primarily or exclusively in the skin. Mycosis fungoides (MF) and its leukaemic variant, Sézary's 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.