Cutaneous T-Cell Lymphoma - Treatment Options and Expectations

Treatment for Mycosis Fungoides, Sezary Syndrome, and More

How is cutaneous T-cell lymphoma treated?. Istockphoto.com/Stock Photo©AlexRaths

How is cutaneous T-cell lymphoma (CTCL) treated, and what are the goals of therapy?

Understanding Cutaneous T-Cell Lymphoma Treatment

It's important in discussing treatment of CTCL to keep in mind that ctcl is not one disease, but several, including mycosis Fungoides ,, Sezary syndrome, reticular cell sarcomas, and more.  If you've been diagnosed you are probably already familiar with the definition, symptoms, and diagnosis of cutaneous t-cell lymphoma, but as a quick review, CTCL is a form of non-Hodgkin's lymphoma that begins in the skin, and is treated quite differently than other lymphomas that primarily affect lymph nodes.

  Chemotherapy that is effective for other types of non-Hodgkin's lymphoma is not ordinarily effective for patients with CTCL.

Treatment options depend on several factors, including the extent of skin involved, the type of lesions, and the involvement of nodes or other organs. Those whose disease is limited to the skin have excellent responses to treatment. For those with visceral organ or node involvement, however, the aim of treatment is palliation (controlling symptoms and improving the quality of life).

Electron beam therapy

Radiation is an effective treatment for CTCL. Electron beams are a type of radiation that treats superficial tissues like the skin but does not penetrate deeply enough to affect other organs. This type of treatment is ideal for CTCL as large areas of the skin need to be treated, without causing radiation damage to bone marrow and several other organs. Unless only a small area of skin is involved, the entire skin of the body is treated with electron beams.

This kind of treatment requires a specialized and experienced facility for treatment. Treatment lasts for several weeks.

Topical chemotherapy (Chemo on the Skin)

Chemotherapy agents like Nitrogen Mustard or Mustargen (mechlorethamine) and BCNU or Gliadel (carmustine) can be used as a treatment for CTCL.

These drugs are available as ointment-based preparations and need to be applied to the skin for several weeks. An allergy-like skin reaction indicates that the drug is effective and is a pointer to the correct dose for each patient. Both agents are effective and lead to the disappearance of disease in the majority of patients, though maintenance treatment may be required to prevent the lesions from coming back.

Phototherapy with UV light

Ultraviolet (UV) light can cause DNA damage in lymphocytes (the cells that turn into lymphomas), but ordinary skin cells are unaffected because they have means to protect themselves from UV light damage. Both UV-A and UV-B light – different in terms of their wavelength – are effective against CTCL. UV-A light is used with a substance called psoralen (this is called PUVA therapy). Psoralen is administered by mouth, or occasionally on the skin dissolved in bath water. Both forms of UV light can clear 60 to 90 percent of skin lesions and some patients may be controlled for a long time or even cured.

Targetin (Bexarotene)

Targetin (bexarotene) belongs to the class of drugs called retinoids. Retinoids are useful for a variety of skin disorders including lymphomas of the skin. Targetin gel is used once or twice a day for one or two months in early stage CTCL with limited skin involvement. A skin inflammation (dermatitis) occurs and the dose is reduced for two more months. Once the drug is stopped the skin lesions reduce. For those with extensive involvement and in patients with relapse of skin lymphoma, oral formulations of Targetin are available. When used alone, oral Targetin has relatively good response rates.

Ontak (Denileukin Difitox)

Ontak (denileukin difitox) is a type of drug called a monoclonal antibody. Monoclonal antibodies are drugs that target a specific molecule on the surface of cells. Ontak (enileukin difitox) is a monoclonal antibody that binds to the receptor for interleukin-2 (IL-2), a substance that activates T-cells to multiply and become cancerous. Attached to the monoclonal antibody is a toxin or poison that kills the cell to which it binds. Ontak binds to T-cells that have become the cancer cells of cutaneous T-cell lymphoma and the toxin kills the cell. It is used mostly as salvage treatment after other treatments have failed.  (A salvage treatment is used to extend life or to decrease symptoms but not as a method to cure cancer.)

Intron A (Interferon alpha)

Intron (interferon alpha) is a biological agent that is used in many conditions including cancers. Though not commonly used for CTCL, it produces good results when used with PUVA therapy or a salvage treatment of this lymphoma. Intron A is used as an injection that is administered subcutaneously (just below the skin).

Chemotherapy infusions or oral pills

Chemotherapy agents are used rarely in CTCL, mainly as a treatment for advanced disease. Chemotherapy may be administered either as infusions in the veins or as oral pills. The most common chemotherapy pills used are methotrexate, Leukeran (chlorambucil) or VePesid (etoposide.) Doxil (pegylated doxorubicin) is the most effective chemotherapy infusion. Some short-term benefit may be gained by chemotherapy to those who have advanced disease that has not responded to other therapies.  It's important to carefully weigh the benefits of these treatments against the side effects of chemotherapy.

Sources:

National Cancer Institute. Mycosis Fungoides and the Sezary Syndrome Treatment – for health professionals (PDQ). Updated 01/29/16. http://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq

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