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T cell lymphoma of the skin

Men and women discussing non Hodgkin's lymphoma

This page tells you about cutaneous T cell lymphoma. You can find the following information

 

A quick guide to what's on this page

Cutaneous T cell lymphoma (CTCL)

CTCL is a rare type of non Hodgkin lymphoma that affects the skin. The 2 most common types are called mycosis fungoides and Sezary syndrome.

Mycosis fungoides is a slow growing (low grade) type of CTCL. It often starts as an irregularly shaped area of dry or scaly skin. It can appear as a single patch or in several areas of the body. These abnormal areas may form scaly raised patches, called plaques. Rarely, the disease becomes more advanced and the skin appears red, swollen and sore all over. This is called erythrodermic mycosis fungoides. In a few cases the cancerous cells can spread to the lymph nodes or other organs such as the liver.

Sezary syndrome is closely related to mycosis fungoides but most or all of the skin is covered in a red itchy rash. There are also cancerous T cells (also called Sezary cells) in the blood. It is a faster growing (high grade) type of CTCL.

Treatment for CTCL

The treatment you have depends on the stage of the lymphoma. This means how much of the skin is affected and if it has spread to the lymph nodes or other body organs. There are 4 main stages for mycosis fungoides – from stage 1 (early disease) to stage 4 (advanced). Sezary syndrome is an advanced stage, as it is a widespread disease at diagnosis.

Many types of CTCL are slow growing and you may not need treatment straight away but will have regular check ups with your specialist. For early stage CTCL, you may have chemotherapy or steroid creams or gels (topical treatments) to put onto the affected area. Other treatments directly to the skin include treatment with ultraviolet light (PUVA or narrow band UVB), low dose radiotherapy, or total skin electron beam therapy (TSEBT). 

Treatments for more advanced disease include chemotherapy (as tablets or into a vein), interferon, a type of retinoid called bexarotene, and extracorporeal photopheresis (ECP). Some young and healthy people with advanced CTCL may have high dose chemotherapy and a stem cell or bone marrow transplant

 

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What cutaneous T cell lymphoma (CTCL) is

CTCL is a rare type of non Hodgkin lymphoma that affects the skin.  A type of cell called a T lymphocyte grows out of control within the skin. Many types of CTCL start as flat red patches on the skin, which can sometimes be itchy. With darker skin, the patches may appear lighter or darker than the surrounding skin. 

In the early stages, the skin patches can look like other common conditions such as eczema or psoriasis. CTCL is diagnosed by taking a sample of the affected skin (a biopsy) and examining it under a microscope for cancerous T cells. Diagnosing skin lymphoma can be difficult and so it is not unusual to have more than one biopsy over a number of weeks or months.

We don't know what causes CTCL. 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 than women.

There are a number of different types of CTCL. Most are slow growing (low grade) but some can be fast growing (high grade). Knowing the type and grade of the lymphoma helps doctors decide on the best treatment and how soon it should start. For some very slow growing types of CTCL, treatment may not need to start straight away.

The most common types of CTCL are mycosis fungoides (pronounced my-coh-sis fun-goyd-eez) and Sezary syndrome.

Mycosis fungoides

About half (50%) of the people diagnosed with CTCL have mycosis fungoides. It is a very slow growing (low grade) type of CTCL. It often starts as an irregular shaped area of dry or scaly skin. It can appear as a single patch or in several areas of the body. The patches can appear anywhere on the body but are more often found on the chest, abdomen, back and buttocks. These abnormal areas of skin may form scaly raised patches, called plaques.

In a small number of people, raised lumps (tumours) can appear. Rarely, the disease becomes more advanced and the skin appears red, swollen and sore all over. This is called erythrodermic mycosis fungoides. In a few cases the cancerous cells can spread to the lymph nodes or organs such as the liver. 

Sezary syndrome

About 1 in 20 people with CTCL (5%) have Sezary syndrome. It is closely related to mycosis fungoides but most or all of the skin is covered in a red itchy rash. There are also cancerous T cells (called Sezary cells) in the blood. It is a faster growing (high grade) type of CTCL. People with Sezary syndrome often have a weakened immune system and so you have an increased risk of infection.

 

The stages of CTCL

The stage shows how much of the skin is affected by lymphoma and if it has spread to the lymph nodes or other body organs. There are 4 main stages for mycosis fungoides. Sezary syndrome is an advanced stage, as it is a widespread disease at diagnosis.

Stage 1 means the lymphoma only affects the skin (patches or plaques). This stage is divided into 2 further groups

  • Stage 1A means that the lymphoma affects less than 10% of the skin
  • Stage 1B means that the patches or plaques have become more widespread, affecting more than 10% of the skin

Stage 2 is divided into 2 groups

  • Stage 2A means there are patches or plaques on the skin. The lymph nodes are swollen but there are no cancerous T cells there
  • Stage 2B means there are one or more lumps (tumours) in the skin

Stage 3 is divided into 2 groups

  • Stage 3A means that most of the skin (more than 80%) appears red and sore (erythrodermic mycosis fungoides)
  • Stage 3B is the same as 3A, but there are some cancerous T cells (Sezary cells) in the blood

Stage 4 is divided into 3 groups

  • Stage 4A1 means there are high numbers of cancerous T cells (Sezary cells) in the blood (Sezary syndrome)
  • Stage 4A2 means there are cancerous T cells in the lymph nodes
  • Stage 4B means the cancer has spread to other organs of the body, such as the liver or spleen

The outlook (prognosis) of CTCL depends on

  • The type of CTCL you have
  • How widespread the lymphoma is
  • Your age and general health

At diagnosis, most people with mycosis fungoides have early stage disease (stage1A, 1B and 2A). Many of these will never grow to the more advanced stages. If you are diagnosed at an early stage, there is a good chance of controlling the disease long term. For many people, it will not affect how long you will live (your life expectancy). You often have long periods of time where you do not need treatment. Occasionally, in some cases of very early stage disease, it may be possible to cure it.

It can be harder to treat advanced mycosis fungoides and Sezary syndrome. With mycosis fungoides there can still be long periods of time when you are symptom free.

 

Treatments for CTCL

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

Watch and wait

Many skin lymphomas are slow growing, so you may not need treatment straight away. You will have regular check ups with your specialist.  Your doctor may suggest using a moisturising cream every day and adding an emollient to the bath water to help with skin dryness and relieve mild itching. Emollients help to prevent loss of water from your skin by creating a protective layer.

Treatment directly on the skin

Treatment directly on to the skin (topical treatment) is helpful for early stage CTCL. Treatments include steroid creams or gels, and chemotherapy cream.

Steroid creams or gels help to relieve itching and can clear up patches or plaques of CTCL for long periods of time. Doctors generally prescribe topical steroids for short periods as they can cause side effects such as skin thinning in the affected area.

Chemotherapy cream is called topical chemotherapy. You apply chemotherapy cream to the affected area daily or every other day. You need to wear gloves when putting the cream on 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. In the UK, doctors most commonly use carmustine (BCNU).

Treatment with ultraviolet light (PUVA)

Psoralen ultraviolet light treatment (PUVA) is very helpful for plaques and larger areas of CTCL. Psoralen (P) is a drug that makes your skin very sensitive to ultraviolet light (UVA) for about 24 hours. 

To have the treatment, you take psoralen tablets about 2 hours before having an ultraviolet light shone onto your skin. Psoralen also makes your eyes more sensitive to light, so on the day you have treatment you will need to wear sunglasses, both indoors and outdoors, until bedtime. Your doctor or nurse will give you advice about protecting your skin and eyes from ultraviolet light and for how long.

You usually have treatment 2 times a week until there is no sign of CTCL or for a maximum of 30 treatments. When treatment finishes, your doctor will monitor you regularly in clinic. CTCL generally comes back and so you may need PUVA treatment again. 

Short term side effects may include skin redness, dry and itchy skin, and a rash. One of the long term side effects of PUVA, particularly with higher doses, is an increased risk of non melanoma skin cancer. Your doctor will weigh up the risks and benefits of treatment and talk you through them. The main concern is to treat your current lymphoma. Your doctor will talk to you about taking care in the sun, and will watch you for signs of skin cancer. Non melanoma skin cancers are treatable.

Narrow band UVB treatment (TLO-1)

This is similar to having PUVA treatment, but it uses UVB light rather than UVA. And you do not need to take a drug that makes your skin more sensitive to light. However,TLO-1 treatment does not go as deep into the skin as PUVA treatment. So you usually only have it to treat patches and very thin plaques of disease.

The side effects of TLO-1 treatment are similar to PUVA, but there is a lower risk of developing non melanoma skin cancer with narrow band UVB treatment.

Radiotherapy

Low dose radiotherapy to the skin (superficial radiotherapy) also works well for CTCL. You are only likely to have radiotherapy for small localised plaques or tumours in the skin. Or you may have it for clearing up patches that remain after PUVA treatment. You usually have between 2 and 5 treatments. 

Radiotherapy to the skin does not have very many side effects. The skin in the treatment area may become slightly red and sore. This will begin to disappear once the treatment is over. If you have radiotherapy to a part of the body that has hair, you will have some temporary hair loss. There is a small increased risk of non melanoma skin cancer in the area.

Total skin electron beam therapy (TSEBT)

You have TSEBT in a specialist centre. You have this type of radiotherapy to larger areas of CTCL that have not grown too deeply into the skin. You usually have TSEBT for CTCL that has not responded to other treatment, or has come back. You have it 4 to 5 days a week for between 2 and 5 weeks. Any areas of your body that are very sensitive or where the skin is thin will be protected. For example, you will have eye shields to protect your eyes.

TSEBT works well. However it does not keep the CTCL under control forever. But you can have treatment a second time when the lymphoma comes back.

You can get side effects 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 nurse and doctor will monitor you closely for this. The main concern is to treat the lymphoma.

Chemotherapy as tablets or into a vein

Doctors normally only use chemotherapy 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. It may help for months rather than years.

You may take chemotherapy as a tablet, such as 

Or you may have it injected through a drip into a vein, for example

You may have one chemotherapy drug or a combination of drugs.

Interferon

Interferon is a type of biological therapy. It works by stimulating your immune system to control the lymphoma. You have interferon as an injection under the skin. You have this 3 times a week. Your nurse can teach you how to give the injection yourself. You may have interferon on its own or with other treatment such as PUVA, extracorporeal photopheresis (ECP) or bexarotene.

The most common side effects are

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

Your nurse will give you paracetamol before you have the interferon injection to help with some of these side effects. There is detailed information about interferon side effects in our cancer drugs section.

Bexarotene (Tagretin)

Bexarotene is a retinoid. Retinoids are a group of drugs related to vitamin A. You take bexarotene as a capsule. You usually have it for more advanced CTCL that has come back after other treatments. You may have it on its own or with another treatment such as 

The main side effects include an increase in cholesterol and fat (triglyceride) levels in your blood, and a drop in thyroid hormones. You will have regular blood tests to check these. You may need to take tablets to keep these levels within normal ranges. 

Extracorporeal photopheresis (ECP)

ECP is a type of PUVA treatment that treats the blood. Doctors use it to treat people with advanced CTCL, especially Sezary syndrome. This treatment is only available in some specialist centres. 

To have the treatment your nurse connects you to a special machine by a drip. Your blood goes through the machine, which separates off some of your white blood cells. Your blood, minus the white blood cells, then goes back into your vein. The separated white blood cells are treated with a light sensitising drug (psoralen) and then exposed to ultraviolet light (UVA). This light activates the drug so that it is able to destroy the lymphoma cells. After this process, your nurse gives back the treated white blood cells into your vein. You usually have the treatment again the following day.

The treatment itself takes between 3 and 4 hours, and you have the 2 day cycle every 2 to 4 weeks. Many people have this treatment for a year or more. Having this procedure does not hurt, but some people may feel a bit weak or dizzy during or after it. Afterwards your skin and eyes may be more sensitive to sunlight for about 24 hours. So you need to protect yourself from the sun and also need to wear sunglasses.You may have ECP with other treatments such as bexarotene or interferon.

Stem cell or bone marrow transplant

A few people with advanced CTCL have treatment with high dose chemotherapy and a stem cell or bone marrow transplant

Stem cells are very early blood cells in the bone marrow that develop into red blood cells, white blood cells and platelets. We need stem cells in order to survive. High dose treatment destroys the stem cells in the bone marrow. So after the high dose treatment you have stem cells or bone marrow into a vein through a drip. Then you can make the blood cells you need again.

For CTCL, you usually have stem cells or bone marrow from another person. This is called an allogeneic stem cell or bone marrow transplant. Doctors usually only suggest this type of treatment to people who are young and fit and are most likely to be able to cope with it.

We have information about stem cell transplants and bone marrow transplants in the treating non Hodgkin lymphoma section.

 

Research into CTCL

Research is looking into

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 people. Treatments researchers are looking at include

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 one randomised trial, 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. Other trials have looked at denileukin for early stage CTCL. Early trials are looking at giving it with other treatments such as bexarotene for advanced disease.

The side effects of this treatment include

  • Flu like symptoms of sickness, aching and a high temperature (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
  • Problems with vision – though this is rare

Monoclonal antibodies

Monoclonal antibodies are a type of biological therapy. There have been some good responses to a drug called alemtuzumab (Campath) in some patients with CTCL. There have been reports of the skin lesions shrinking or even disappearing altogether for a time after the treatment. Early trials have been looking at another type of monoclonal antibody called zanolimumab, with promising results.

Lenalidomide (Revlimid)

Lenalidomide is a type of biological therapy that helps the body’s immune system target cancer cells. One trial has been looking at lenalidomide after chemotherapy for CTCL. Chemotherapy can shrink areas of CTCL but it often starts to grow again. So researchers want to find out whether having lenalidomide after chemotherapy can prevent or delay the CTCL coming back. We are waiting for the results of this trial to be published.

HDAC inhibitors

Histone deacetylase (HDAC) inhibitors block enzymes (histone deacetylases) in the body which cells need to grow and divide. Blocking these enzymes may stop cancer growing. Vorinostat and romidepsin are 2 HDAC inhibitors that have been looked at in trials. Early trials have shown responses in about a third of patients with CTCL. Panobinostat has also been looked at in early trials with promising results.

Side effects of these treatments include

A trial is looking at a new HDAC inhibitor called JNJ-26481585 for cutaneous T cell lymphoma that has come back or got worse despite having other treatment. The trial aims to find out whether JNJ-26481585 helps people with CTCL and how it affects their quality of life. The people taking part in this trial are now being followed up and we are waiting for the results.

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 feel about 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. This study is now closed and we are waiting for the results.

 

More about NHL

We have detailed information about non Hodgkin lymphoma treatments in this section.

If you would like more information about any aspect of non Hodgkin lymphoma, you can phone the Cancer Research UK nurses on freephone 0808 800 4040. The lines are open from 9am to 5pm, Monday to Friday. They will be happy to answer any questions that you have.

Our non Hodgkin lymphoma organisations page gives details of other people who can provide information about NHL and its treatment. Some organisations can put you in touch with a cancer support group. They often have free factsheets and information which they can send to you. There are also books, booklets, CDs and other resources available about non Hodgkin lymphoma. Some of these are free. Look at our NHL reading list for details.

If you want to find people to share experiences with online, you could use Cancer Chat, our online forum.

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Updated: 1 September 2014