Implications for practice

• Although there are few well-designed RCTs in CTCL, there is convincing evidence that several skin-directed therapies have a significant therapeutic effect. However, there is a fundamental lack of data on the impact of different therapies on DFS and OS, which will only become clearer when the results of key RCTs in different stages of disease become available.

• In addition, patients with early-stage disease can have a normal life expectancy, and so aggressive therapies with a significant mortality and morbidity should be avoided in these patients, especially when the chance of a cure is very low.

• Patients with early-stage disease (IA/IB/IIA) should be offered skin-directed therapies such as topical mechlorethamine, phototherapy, PUVA and superficial radiotherapy. Alfa interferon should be considered for patients with persistent or recurrent stage IB/IIA disease. Some patients with stage IA disease may not require any specific therapy.

• Patients with late stages of disease (IIB/IV) should be offered TSEB, single-agent palliative chemotherapy and multi-agent chemotherapy, according to performance status.

• Patients with erythrodermic disease should be offered photopheresis, immunotherapy and single-agent chemotherapy as palliative therapy aiming to improve quality of life. TSEB therapy may be indicated for erythrodermic disease when there is a lack of significant peripheral blood tumour burden.

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