Highlights & Basics
- 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 medications, 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.
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Citations
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Willemze R, Hodak E, Zinzani P, et al; ESMO Guidelines Working Group. Primary cutaneous lymphomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29(4 suppl):iv30-40.[Abstract][Full Text]
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: primary cutaneous lymphoma [internet publication].[Full Text]
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