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Treatment

Treatment for cutaneous T cell lymphoma (CTCL) varies depending on the presentation and extent of the disease. The stage of the CTCL affects the prognosis and guides therapeutic decisions. In early stages of disease, more options are available and cures may be achieved. Treatment is typically directed to the whole body, because there may be undetected lesions that should be treated. These lesions may temporarily become more apparent during therapy and then resolve.

Phototherapy may be useful in the treatment of CTCL, which involves exposure to controlled, progressive ultraviolet radiation. Either ultraviolet B (UVB) or UVA rays are used. An oral medication called Psoralen is used with UVA ray phototherapy, because the drug makes the skin more responsive to UVA rays. This combination therapy is also referred to as PUVA. Patients receiving UVB or PUVA are treated 3 times per week, until remission is achieved. At that point, treatments are tapered off, although monthly maintenance therapy may be continued for years. Phototherapy, particularly with PUVA, can increase one s risk for other types of skin cancer, and all patients should be checked periodically. Specialized glasses must be worn for 24 hours following each treatment, because Psoralen causes increased sensitivity to light.

Topical nitrogen mustard (a chemotherapeutic agent) can also be used for patch or plaque stage treatment. The dose of the medication, which the patient applies to his or her body, is gradually increased as tolerance develops. This therapy can induce long-term remission and even cures. Nitrogen mustard therapy can be used in combination with phototherapy to increase effectiveness.

Electron-beam therapy is a widely accepted treatment option. With electron-beam therapy, radiation affects the skin only and does not penetrate to the internal organs, such as the intestines or bone marrow. This greatly limits side effects.

Whole body electron-beam radiation results in complete remission in about 84% of individuals. Relapse of the cancer is seen more often in more advanced disease. This therapy is usually not a first choice, as the allowable lifetime amount of radiation is limited. Thus, the therapy is usually reserved for patients who do not respond to other forms of treatment.

Extracorporeal photochemotherapy (ECP) involves removing a portion of the patient's white blood cells from the body, treating them with UVA light, and returning them to the patient s body. These treatments are given on 2 consecutive days, once a month. This helps to treat and manage erythrodermic CTCL. While not all of the white blood cells respond to this treatment, even a partial response can make the disease more manageable and improve the patient's quality of life.

Chemotherapy is used to treat CTCL, but it is typically used only to alleviate pain. To date, no chemotherapeutic regimen has been curative in advanced cases of the disease.

Prevention
The etiology (cause) of CTCL is not well understood. In some cases, a virus has been responsible for development of this disease and exposure, when known, should be limited.

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