Since primary cutaneous ALCL rarely spreads beyond the skin and is usually associated with an excellent prognosis, most solitary lesions can be managed with local treatment, surgical excision or local radiotherapy. Other therapies include psoralin UV-A (PUVA) or UV-B phototherapy. Single agent anti-cancer chemotherapy is occasionally used either locally (such as topical nitrogen mustard) or systemically (for example, low dose methotrexate). Two additional drugs were approved in the USA for the treatment of cutaneous T-cell lymphoma . Targretin® (bexarotene) is an oral therapy reported to produce a partial response in cutaneous T-cell lymphomas in approximately 30% of patients but complete responses in less than 2%. Specific figures of primary cutaneous ALCL are not listed on the prescribing information.

Ontak® (denileukin diftitox) was also approved in the USA for the treatment of cutaneous T-cell lymphoma. The drug, which is a combination of diphtheria toxin and a ligand that binds to a specific target (the IL2 receptor) on the surface of the lymphoma cells, must be given intravenously. Studies demonstrated 30% of the patients' tumors were reduced 50% or more with Ontak, with the response lasting an average of 4 months. Ten percent of the patients achieved a complete clinical remission that lasted an average of 9 months. Because Ontak also affects normal lymphocytes, which are infection-fighting cells, and because patients with cutaneous T-cell lymphoma are prone to infections, patients should be monitored carefully during treatment. Severe infections were reported in 23% of patients in the trial, although it is unclear whether Ontak was the cause. Other side-effects related to the intravenous infusion of Ontak include allergic reactions, flu-like symptoms and fluid retention that may lead to other complications. Response rates for primary cutaneous ALCL are not listed on the prescribing information for Ontak.

In addition, there are a number of new therapies in clinical development for the treatment of ALCL.