T cell lymphoma of the skin is also called cutaneous T cell lymphoma (CTCL). Find out more about what it is, the different types and how it is treated.
What it is
Cutaneous T cell lymphoma (CTCL) is a rare type of non Hodgkin lymphoma (NHL) that affects the skin.
T lymphocytes are a type of white blood cell. These T cells grow 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.
Doctors diagnose CTCL by taking a sample of the affected skin (a biopsy). A specialist doctor (pathologist) examines it under a microscope, looking for cancerous T cells.
Diagnosing skin lymphoma can be difficult. So it is not unusual to have more than one biopsy over a number of weeks or months.
There are 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 might 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.
About half (50%) of people diagnosed with CTCL have mycosis fungoides. It is a very slow growing (low grade) type of CTCL.
Mycosis fungoides 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 might 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.
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.
Sezary syndrome is a faster growing (high grade) type of CTCL. People with Sezary syndrome often have a weakened immune system. So there is an increased risk of infection.
The stage shows how much of the skin is affected by lymphoma, and whether 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 because it is a widespread disease at diagnosis.
Stage 1 only affects the skin (in patches or plaques). This stage is divided into two 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 and 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
As for most cancers, treatments depend on the stage of the disease when it is diagnosed. For some very slow growing types of skin lymphoma, treatment may not need to start straight away.
Many skin lymphomas are slow growing, so you might not need treatment straight away. Doctors call this watch and wait.
You 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 to the skin is also called topical treatment. It is helpful for early stage CTCL. Treatments include steroid creams or gels, and chemotherapy cream.
Steroid creams or gels
These 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.
This is called topical chemotherapy. In the UK, doctors most commonly use a chemotherapy drug called carmustine (BCNU).
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.
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.
How you have it
To have the treatment, you take psoralen tablets. About 2 hours later the doctor shines an ultraviolet light onto your skin.
You usually have treatment 2 times a week until there is no sign of lymphoma. Or you have it for a maximum of 30 treatments.
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.
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.
This is similar to 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.
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 uses high energy x-rays to kill cancer cells. Low dose radiotherapy to the skin (superficial radiotherapy) can work well for CTCL.
When you have it
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 treatment area.
This is a type of radiotherapy. You have it in a specialist centre.
When you have it
You have TSEBT to larger areas of CTCL that have not grown too deeply into the skin. You usually have it 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 uses anti cancer (cytoxic) drugs to destroy cancer cells. You can have it as tablets or into a vein (intravenously).
When you have it
Doctors normally only use chemotherapy when CTCL is advanced. You have it 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 might take one of the following chemotherapy drugs as a tablet:
Or you might have chemotherapy through a drip into a vein. Drugs include:
- pentostatin (Nipent)
- liposomal doxorubucin
You might have one chemotherapy drug or a combination of drugs.
Interferon is a type of biological therapy. It works by stimulating your immune system to control the lymphoma.
How you have it
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:
- aches and pains
- sadness or depression
Your nurse will give you paracetamol before you have the interferon injection to help with some of these side effects.
Bexarotene is a retinoid. Retinoids are a group of drugs related to vitamin A.
When you have it
You usually have it for more advanced CTCL that has come back after other treatments.
How you have it
You take bexarotene as a capsule. You may have it on its own or with another treatment such as:
- psoralen ultraviolet light treatment (PUVA)
- extracorporeal photopheresis (ECP)
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.
ECP is a type of PUVA treatment that treats the blood.
When you have it
Doctors use it to treat people with advanced CTCL, especially Sezary syndrome. This treatment is only available in some specialist centres.
How you have it
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 nurse or doctor treats the separated white blood cells with a light sensitising drug (psoralen). They then expose them 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.
You may have ECP with other treatments such as bexarotene or interferon.
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.
These are high dose treatments used for some types of cancer.
When you have it
A few people with advanced CTCL have treatment with high dose chemotherapy and a 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.
How you have it
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.
Before a transplant, you have high dose treatment to destroy the stem cells in the bone marrow. After the high dose treatment you have stem cells or bone marrow into a vein through a drip. Then your body makes 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.
Researchers are looking at treatment for CTCL and how people with CTCL are cared for.
Doctors are testing newer treatments for cutaneous T cell lymphomas. They are looking for treatments that can control it for longer, and hopefully get rid of it altogether in more people. They are looking at the following treatments:
This 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.
These are a type of biological therapy. There have been some good responses to a drug called alemtuzumab (Campath) in some patients with CTCL. And early trials have looked at another type of monoclonal antibody called zanolimumab, with promising results.
This is a type of biological therapy that helps the body’s immune system target cancer cells. Doctors have been looking at whether having lenalidomide after chemotherapy can prevent or delay the CTCL coming back.
Histone deacetylase (HDAC) inhibitors block enzymes (histone deacetylases) in the body which cells need to grow and divide. Doctors have looked at HDAC inhibitors for CTCL including:
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. A study is looking at 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.