Cutaneous T cell lymphoma

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)
• Sezary syndrome.

Mycosis fungoides is a low grade (indolent) type of NHL. It often starts as an area of red, inflamed skin which can spread to other areas and may form raised patches, called plaques. Doctors generally think that Sezary syndrome is an advanced form of mycosis fungoides. In Sezary syndrome, most of the skin is covered in a red itchy rash, the lymph nodes are affected and there are abnormal T cells 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 very 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 cells in 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. You put these on daily. You need to wear gloves because the chemotherapy drug harms normal skin. Some people develop an allergic reaction to the chemotherapy and have to stop treatment for a while, or change 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.

You have treatment 3 times a week at first. Then, if the skin lesions clear up, you may have it twice a week. Once there is no sign of the CTCL (this means that it is completely in remission) you have weekly treatment to keep it under control. 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 to 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

Radiotherapy works well for CTCL. You are only likely to have radiotherapy 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. You have 10 to 15 daily treatments. Most people respond well to radiotherapy for CTCL.

Electron beam therapy (EBT)

You have electron beam therapy in a specialist centre. You can have this type of radiotherapy to larger areas of skin. It only affects the skin, so there are fewer side effects than with widespread radiotherapy. The treatment is given 4 days a week for 9 weeks. Any areas of your body which are very sensitive or where the skin is thin will be protected. For example, you will have eye shields to protect your eyes.

EBT works well. It does not keep the CTCL under control for ever. But you can have treatment a second time (or even a third) when the lymphoma 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 your doctor will watch you closely for any signs of other skin cancers. The main concern is to treat the current lymphoma.

Chemotherapy as tablets or into a vein

Chemotherapy is normally only used when CTCL is advanced. You have this to control symptoms and make you more comfortable. Chemotherapy often works well for CTCL, but unfortunately the response does not usually last very long (months rather than years).

Research is looking into

• Treatments for CTCL and
• How people with CTCL are cared for

Treatments for CTCL

Newer 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 (Campath)
• Bexarotene (Tagretin)
• Romidepsin (Istodax)
• Lenalidomide (Revlimid)
• JNJ-26481585

Interferon

Interferon is a type of biological therapy. It is being tried on its own and in combination with other treatments for CTCL. You have interferon as an injection under the skin. There is detailed information about interferon side effects elsewhere on our website. The most common side effects are

• Fever
• Shivering
• Aches and pains
• Tiredness
• Sadness or depression

Denileukin (Ontak)

Ontak (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 – a drop in blood pressure, breathlessness or wheezing, chest and back pain, and leaking of fluid from the small veins into the tissues

Alemtuzumab (Campath)

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 (Tagretin)

Bexarotene is a retinoid. Retinoids are a group of drugs related to vitamin A. You take bexarotene as a tablet. Early trials showed that the CTCL responded in about half the people treated. 

The EORTC 21011 trial looked at bexarotene and PUVA treatment for mycosis fungoides. The researchers wanted to find out whether bexarotene and PUVA is better than PUVA on its own. The trial team found that PUVA with bexarotene worked just as well as PUVA alone and the side effects were acceptable. The researchers noted a trend towards people needing fewer PUVA sessions in the bexarotene group. But they could not rule out this happening by chance because not enough people took part in the trial. So it was not statistically significant. 

The GemBex trial is looking at how well bexarotene and the chemotherapy drug gemcitabine work for CTCL that has got worse, despite having had treatment to the skin, and systemic treatment like interferon or chemotherapy.

Lenalidomide (Revlimid)

Lenalidomide is a type of biological therapy that helps the body’s immune system target cancer cells. A trial is testing lenalidomide after chemotherapy for CTCL. Chemotherapy can shrink areas of CTCL but it often starts to grow again. Researchers want to find out whether having lenalidomide after chemotherapy can prevent or delay the CTCL coming back.

JNJ-26481585

One trial is testing a new biological therapy drug called JNJ-26481585 for cutaneous T cell lymphoma. It is for people with CTCL that has come back or got worse despite having other treatment. JNJ-26481585 blocks substances (enzymes) in the body called histone deacetylases (pronounced dee-as-et-isle-azes). Cells need these to grow and divide. Blocking them may stop cancer growing. Drugs that block these enzymes are called histone deacetylase inhibitors (HDAC inhibitors). The trial aims to find out whether JNJ-26481585 helps people with CTCL and how it affects their quality of life.

How people with CTCL are cared for

A study is looking at how people with CTCL are cared for. Researchers know that some people are treated for T cell lymphoma of the skin for many years. They want to find out how people experience their treatment and care. The study aims to find out about the care and support needs of people with T cell lymphoma of the skin. It also wants to find out about the needs of their carers.