Cutaneous T cell lymphoma

• A quick guide to what's on this page
• What cutaneous T cell lymphoma (CTCL) is
• The stages of CTCL
• Treatments for CTCL
• Newer treatments

CTCL is a rare type of lymphoma that affects the skin. Uncontrolled T cells grow within the skin. The most common types of CTCL are

• mycosis fungoides (pronounced my-coh-sis fun-goyd-eez)
• Reticulum cell sarcoma of the skin
• Sezary syndrome.

In Sezary syndrome, large areas of skin, or lymph glands, are affected, and abnormal T-lymphocytes are also found in the blood.

CTCL is a rare condition with no known cause. There are only about 4 cases diagnosed for every million people in the population. Most people with CTCL are between 40 and 60 years old. It is twice as common in men and slightly more common in black people. It can only be definitely diagnosed by taking a sample of the affected skin (a biopsy) and examining it under a microscope for cancerous T cells.

There are 4 main stages of this condition

• Stage 1 - the disease only affects the skin
• Stage 2 - lymph nodes are enlarged, but there is no sign of cancer in them
• Stage 3 - there are lymphoma cells in the lymph nodes
• Stage 4 - the lymphoma has spread to body organs

There are 3 parts to stage 1 - stages 1A, 1B and 1C. In stage 1A, there are a few scaly red patches on the skin. Less than 10% of the skin is affected. The skin patches can be incredibly itchy.

Stage 1B means the patches have become more widespread, with more than 10% of the skin affected.

In stage 1C, the red itchy patches have turned into raised tumours on the skin that may break down to become sores.

In the advanced stage, there are also abnormal T cells in the blood. At this stage, large areas of skin can become very reddened, painful and swollen. You may have swollen lymph nodes or a swollen liver or spleen.

The outlook (prognosis) of CTCL depends on how widespread the disease is. If less than 10% of your skin is affected, there is a good chance of controlling the disease long term, or of curing it completely.

If more than 10% of your skin is involved, or if you have lymphoma spread to the lymph nodes or a body organ, then this disease is unlikely to be cured. But it can often be controlled for a long time with treatment.

As for most cancers, this depends on the stage of the disease when it is diagnosed. Treatments include

• Chemotherapy on the skin
• Treatment with ultraviolet light (PUVA))
• Radiotherapy
• Electron beam therapy (EBT)
• Chemotherapy as tablets or into a vein

Chemotherapy on the skin

This is called 'topical chemotherapy'. Early CTCL is most often treated with chemotherapy creams or lotions. These are put onto the skin daily. You need to wear gloves to do this as the chemotherapy drug will harm normal skin. Some people develop an allergic reaction to the chemotherapy and have to stop treatment for a while, or switch to another drug.

Treatment with ultraviolet light (PUVA)

Another widely used treatment is PUVA. This stands for psoralen ultraviolet treatment. Psoralen is a drug that makes your skin very sensitive to ultraviolet light. To have the treatment, you take a psoralen tablet 1 to 2 hours before having an ultraviolet light shone onto your skin.

The treatment is given 3 times a week at first. Then, if the skin lesions clear up, it can be reduced to twice a week. Once the CTCL is completely in remission (that means there is no sign of it) you have weekly treatment to keep it at bay. This is called maintenance therapy and continues for a year.

Often doctors recommend that you have PUVA every 2 weeks for a year after that. And every 3 weeks for another 2 years. After this time, you can stop treatment altogether. But the CTCL can still come back and you may be asked to have treatment every 4-6 weeks for a bit longer. Unfortunately it can still come back after that. This condition is not considered cured until you have been free of it for 5 years after you finished all treatment.

Radiotherapy

CTCL responds well to radiotherapy. But this treatment is only usually used for small localised areas of CTCL in the skin. Or for clearing up patches that are still there after PUVA treatment. If large areas were treated, you would have too many side effects. In particular, the radiotherapy could kill off too many of your healthy white blood cells. The radiotherapy is given in 10 to 15 daily treatments. Most people respond well to radiotherapy for CTCL.

Electron beam therapy (EBT)

This treatment can only be given in a specialist centre. It is a type of radiotherapy that can be given to larger areas. This is because the electrons can only travel up to 1cm deep into the body. It only affects the skin, so there are fewer side effects than with widespread radiotherapy. This treatment is used for CTCL affacting large areas of skin. So all your skin is treated. The treatment is given 4 days a week for 9 weeks. You are given eye shields to protect your eyes. And your hands and feet are protected for half the course of treatment.

This is a very effective treatment. It does not keep the CTCL under control for ever. But you can have treatment a second time (or even a third) when the disease comes back.

Side effects can occur up to 6 months after the treatment. They can include

• Itching, peeling skin
• Hair loss
• Reddening and swelling of the skin
• Reduced sweating
• Dryness of the skin

In the long term, there is an increased risk of developing other types of skin cancer. But this is less of a risk to you than the CTCL as they can usually be more easily treated. And your doctor will watch you closely for any signs of other skin cancers.

Chemotherapy as tablets or into a vein

This is normally only used when CTCL is advanced. It is given to control symptoms and make you more comfortable. CTCL does respond well to chemotherapy, but unfortunately the response does not usually last very long (months rather than years).

Other new treatments are being tested for cutaneous T cell lymphomas. Doctors are working to find treatments that can control it for longer, and hopefully get rid of it altogether in more patients. Treatments being researched include

• Interferon
• Denileukin (Ontak)
• Alemtuzumab (MabCampath)
• Bexarotene
• Depsipeptide (FK228)

Interferon

Interferon is a type of biological therapy. It is being tried on its own and in combination with other treatments for CTCL. Interferon is given as an injection under the skin. There is detailed information about interferon side effects elsewhere in CancerHelp UK. The commonest side effects are

• Fever
• Shivering
• Aches and pains
• Tiredness
• Low mood or depression

Denileukin (Ontak)

Ontak or denileukin is a combination of a biological therapy called IL-2 and a poison called diptheria toxin. The idea is that the IL-2 finds the cancer cells and the diptheria poison kills them. In trials, about 1 in 3 people responded to this drug. These people had CTCL that had not responded to chemotherapy, so this is promising. The average response lasted about 7 months. But some people had a response for over 3 years. The side effects of this treatment are

• Flu-like symptoms of sickness, aching and fever
• Reactions to the infusion - drop in blood pressure, breathlessness or wheezing, chest and back pain and leaking of fluid from the small veins into the tissues

Alemtuzumab (MabCampath)

Alemtuzumab is a monoclonal antibody. There have been some good responses to alemtuzumab in patients with CTCL. There have been reports of the skin lesions shrinking or even disappearing altogether for a time after treatment with this drug.

Bexarotene

Bexarotene is a retinoid. These are a group of drugs related to vitamin A. Bexarotene is taken as a tablet. Early trials showed that the CTCL responded in about half the people treated. A trial in the UK is currently looking at Bexarotene and PUVA treatment for mycosis fungoides. The researchers want to find out whether bexarotene and PUVA is better than PUVA on its own. They also want to find out which of these treatments has the fewest side effects. Another trial is looking at how well bexarotene and the chemotherapy drug gemcitabine work for CTCL that has progressed, despite having had treatment to the skin, and systemic treatment like interferon or chemotherapy. You may be able to join this trial if you are in this position, and you have stage 1b, 2, 3 or 4a CTCL.

While they are open these trials will be listed on our clinical trials database. Choose lymphoma (non Hodgkin's) from the drop down list of cancer types.

Depsipeptide (FK228)

Another trial has looked at a new drug called depsipeptide (also called romidepsin or FK228). Researchers have been studying it as a treatment for people with CTCL that has come back after previous treatment with chemotherapy or interferon. Early results showed that it could help some people with CTCL. This trial is no longer recruiting patients, and we are waiting for the full results.