Skin lymphoma - cutaneous T cell lymphoma (CTCL)
Skin (cutaneous) lymphoma is a rare type of non-Hodgkin lymphoma (NHL).
NHL is a type of blood cancer that affects white blood cells called 
. It is a cancer of the .
Skin lymphoma is also called cutaneous lymphoma. There are 2 main types of skin lymphoma:
- cutaneous T cell lymphoma (CTCL)
- cutaneous B cell lymphoma (CBCL)
CTCL is the most common type of skin lymphoma. CBCL is more unusual. This page is about CTCL.
The most common types of CTCL are mycosis fungoides (MF) and Sezary syndrome.
What is skin lymphoma?
Skin (cutaneous) lymphoma is a rare type of non-Hodgkin lymphoma (NHL).
Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system.
The lymphatic system is an important part of our immune system. It has tubes that branch through all parts of the body.
These tubes are called lymph vessels or lymphatic vessels and they carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.
There are 2 types of lymphocytes:
-
T cells
-
B cells
In cutaneous lymphoma the T cells or B cells grow out of control within the skin. There are 2 main types of skin lymphoma:
-
cutaneous T cell lymphoma (CTCL) starts in the T cells of the skin
-
cutaneous B cell lymphoma (CBCL) starts in the B cells of the skin
CTCL is the most common type of skin lymphoma. It causes flat red patches on the skin that look like eczema and can be itchy. Several parts of the body can be affected.
CBCL is a more unusual type. People tend to have lumps on their skin in 1 or 2 areas, rather than affecting all of the body.
Cutaneous T cell lymphoma
CTCL is rare. Only around 150 people are diagnosed with CTCL in the UK each year.
Symptoms
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.
Getting diagnosed
Doctors diagnose CTCL by taking a sample of the affected skin. This is called 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.
If your doctor diagnoses lymphoma, you might have further tests. These might include:
- blood tests
- a
CT scan or PET-CT scan - a
lymph node biopsy
You might see different types of doctors during your diagnosis and treatment. There will be a team of people () from different hospital departments involved in your diagnosis. This might include:
- a dermatologist (specialist skin doctor)
- a haematologist (a specialist doctor who treats diseases affecting the blood)
- an oncologist (a cancer specialist)
Types and grades of cutaneous T cell lymphoma (CTCL)
Grade
Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:
- low grade (slow growing)
- high grade (fast growing)
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.
Types
The most common types of CTCL are mycosis fungoides (pronounced my-coh-sis fun-goyd-eez) and Sezary syndrome.
Mycosis fungoides (MF)
Mycosis fungoides (MF) is a very slow growing (low grade) type of CTCL.
MF 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, , 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.
Sezary syndrome
A rare type of CTCL is called 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.
Other rare types
There are some other rare subtypes of CTCL. Your doctor will tell you more about what type you have. And they can tell you whether your subtype is usually slow or fast growing.
Stages of cutaneous T cell lymphoma (CTCL)
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
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
As a rough guide, 10% of your skin is about the same amount of skin as 10 palms of your hand.
Stage 2
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
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
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
SWAT score
You might also hear your doctor talking about a SWAT score. SWAT stands for the Severity Weighted Assessment Tool (SWAT). Your doctor examines each area of lymphoma. They measure how much of your skin is affected by the lymphoma and calculate a score. This is different to your stage. Your doctor might use this score to monitor your CTCL.
Treatment for CTCL
The aim of treatment is to control your skin lymphoma and symptoms for as long as possible, and with as few side effects as possible.
Your treatment depends on:
- the type of skin lymphoma you have
- how advanced it is (the stage)
- your symptoms
- your general health
You often need to try more than one type of treatment over your life time. You might have a single treatment, or several treatments together.
Active monitoring
If you have stage 1 or 2A mycosis fungoides (MF), you may not start treatment straight away. Your doctor monitors you closely. This is called active monitoring or watch and wait. You start treatment if your lymphoma gets worse, or if you have symptoms that are bothering you.
Starting treatment earlier will not affect your outlook (prognosis) or how fast the MF grows. Early stage treatment is more about controlling any symptoms you have.
First treatment for mycosis fungoides (MF)
You might have one or more of the following treatments:
- treatments applied directly to your skin (topical treatment) - these include steroid or
chemotherapy creams - light treatments (phototherapy) - these include psoralen ultraviolet light treatment (PUVA ) and narrow band UVB treatment
radiotherapy to treat small areas of skin lymphoma (localised radiotherapy)
If you have stage 2B or stage 4 MF, you might have a type of radiotherapy called total skin electron beam therapy (TSEBT) as your first treatment.
For stage 3 MF you might have a type of light treatment called extracorporeal photopheresis (ECP).
If your MF is above stage 1a and not responding to , you might also have one of the following drug treatments. You either have these on their own or together with other treatments:
- an
immunotherapy drug called interferon - a
retinoid drug called bexarotene - a
chemotherapy drug (for stage 3 or 4)
Treatment if mycosis fungoides (MF) doesn't go away or comes back
Lymphoma that does not go away with treatment is called refractory lymphoma. Lymphoma that comes back after treatment is called relapsed disease.
Treatment can control MF very well. But it is common for MF to relapse
For stage 1 and 2A MF that comes back you might have:
- a type of radiotherapy called total skin electron beam therapy (TSEBT)
- drug treatment with interferon or bexarotene - you have these on their own or together with light treatments (phototherapy)
For more advanced MF (stage 2B or above) you might have:
- a targeted immunotherapy drug such as brentuximab or mogamulizumab
- chemotherapy
stem cell transplant
You might have a type of radiotherapy called total skin electron beam therapy (TSEBT) for stage 3 MF that comes back.
Treatment for sezary syndrome
You might have these a single treatment, or several treatments together:
- a type of light treatment called extracorporeal photopheresis (ECP) - you have it on its own or with either interferon, methotrexate or bexarotene
- PUVA light treatment with either interferon, methotrexate or bexarotene
- a chemotherapy drug treatment, usually with just one drug
If treatment doesn't work, or your sezary syndrome gets worse again, you might have:
- a targeted immunotherapy drug such as brentuximab or mogamulizumab
- chemotherapy drug treatment
- a stem cell transplant
Types of treatment
We have more information about these treatments below.
Many skin lymphomas are slow growing, so you might not need treatment straight away. Doctors call this active monitoring or watch and wait.
Your doctor may suggest washing with a moisturising cream (emollient) or adding an emollient to the bath water. This is to help with skin dryness and relieve mild itching. Emollients help to prevent loss of water from your skin by creating a protective layer.
It can be difficult to find out you have lymphoma and to not start treatment straight away. But treatment has side effects, so doctors don't want to give people treatment they don't need. You have regular check ups with your specialist.
Unlike other types of lymphoma, you can see your skin lymphoma. So you will know if it is changing or progressing. You can contact your doctor between appointments if you have concerns about this.
If your symptoms or lymphoma are getting worse, you can start treatment.
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.
Chemotherapy cream
This is called topical chemotherapy. In the UK, doctors most commonly use chlormethine gel
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.
Tacrolimus cream
This is a type of cream that acts on your . It blocks a chemical which causes inflammation. This reduces skin inflammation.
Your doctor will tell you about how to apply the cream and what side effects you might have.
Light treatment (phototherapy) works well for skin lymphomas. Ultraviolet (UV) light is a type of light treatment. Doctors use it to treat a number of skin conditions.
The sun produces a number of different ultraviolet UV rays, each with their own wavelength. Ultraviolet A (UVA) and Ultraviolet B (UVB) are both types of rays, or wavelengths. There are different types of UV light treatment for CTCL. You might have:
- psoralen ultraviolet light treatment (PUVA)
- narrow band UVB treatment (TLO-1)
Psoralen ultraviolet light treatment (PUVA)
Doctors use psoralen ultraviolet light treatment (PUVA) to treat 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. You usually take psoralen as tablets on the treatment day. About 2 hours later, you stand in a cabinet which shines ultraviolet light on to your skin.
Instead of taking psoralen as a tablet, you can sometimes have topical PUVA. This means you apply psoralen directly to the skin before UVA exposure. You might apply the psoralen by bathing or soaking part of your body in it. Or you by applying it as a gel directly to the affected areas of skin.
Your doctor will tell you which method is best for you.
You usually have treatment 2 times a week until your skin lymphoma clears. Or you have it for a maximum of 30 treatments.
Oral psoralen also makes your eyes more sensitive to light, so on the day you have treatment you will need to wear sunglasses. You might need to wear these indoors if you are near bright windows, and you wear them outdoors until it is dark. Your doctor or nurse will give you advice about protecting your skin and eyes from ultraviolet light and for how long.
Short term side effects may include skin redness or dry and itchy skin. Tell your doctor or specialist nurse if you have any of these side effects.
One of the long term side effects of PUVA, particularly with higher doses, is an increased risk of non melanoma skin cancer. The risk depends on how many treatments you have. Your doctor will weigh up the risks and benefits of treatment and talk you through them.
Your doctor will also 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 (TLO1)
This uses UVB light. The machine looks similar to PUVA but you do not need to take a drug that makes your skin more sensitive to light.
TLO1 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.
You have treatment 2 or 3 times a week until your skin lymphoma clears. You have up to a maximum of 30 treatments.
Short term side effects may include skin redness, dry and itchy skin, and a rash. In the long term, continuous or long term treatment might increase your risk of developing non melanoma skin cancer. But your doctor usually only offers a one off course of treatment a year for skin lymphoma. So this might not affect your skin cancer risk. Your doctor will talk to you about how this treatment affects your own risk of skin cancer. This will depend on your situation.
Radiotherapy uses high energy x-rays to kill cancer cells. Low dose radiotherapy to the skin (superficial radiotherapy) can work well for CTCL.
You might 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. Doctors also call this localised radiotherapy.
Radiotherapy to the skin does not have very many side effects. The skin in the treatment area may become slightly red and sore. 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.
Total skin electron beam therapy (TSEBT)
This is a type of radiotherapy. You have it in a specialist centre.
You have TSEBT to larger areas of CTCL that have not grown too deeply into the skin. You might have it if CTCL has not responded to other treatment, or has come back. TSEBT works well. But it doesn't keep the CTCL under control forever. But you can have treatment a second time when the lymphoma comes 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.
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.
Bexarotene is a retinoid. Retinoids are a group of drugs related to vitamin A. You might have it for more advanced CTCL or when it has come back after other treatments. You have it on its own or with another treatment.
You take bexarotene as a capsule.
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 take tablets to keep these levels within normal ranges.
Your doctor might suggest you try another type of retinoid drug called acitretin. This drug is usually used for psoriasis and doesn't have a for treating CTCL. It doesn't affect your thyroid gland in the same way as bexarotene.
Targeted cancer drugs work by targeting the differences in cancer cells that help them to grow and survive. Other drugs help the immune system to attack cancer. These are called immunotherapy.
Some drugs work in more than one way. So they are targeted as well as working with the immune system.
Interferon (Peginterferon alfa 2a)
Interferon is a type of immunotherapy. You have interferon as an injection under the skin. You might have this once a week. You may have interferon on its own. Or together with other treatments.
Interferon is also called peginterferon alfa 2a or Pegasys.
Brentuximab and mogamulizumab
Brentuximab and mogamulizumab are both types of These drugs target specific proteins on cancer cells.
Brentuximab targets a protein called CD30. Mogamulizumab targets a protein called CCR4 on T cells.
You might have one of these treatments for more advanced CTCL if you have already tried another drug treatment. And if your lymphoma has come back or is still growing.
Chemotherapy uses anti cancer (cytoxic) drugs to destroy cancer cells. You can have it as tablets or into a vein (intravenously).
You usually only have chemotherapy if your 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.
Types of chemotherapy
You might take one of the following chemotherapy drugs as a tablet:
- chlorambucil
- etoposide
Or you might have chemotherapy through a drip into a vein. Drugs include:
- gemcitabine
- pentostatin (Nipent)
- fludarabine
- liposomal doxorubucin
You might have one chemotherapy drug or a combination of drugs.
Extracorporeal photopheresis (ECP) uses ultraviolet light. But unlike PUVA, it treats your white blood cells, not just your skin.
You might have it for advanced CTCL, or for Sezary syndrome. It 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.
Side effects
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.
A few people with advanced CTCL have a stem cell 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 the side effects.
You can have a stem cell transplant after very high doses of chemotherapy. The chemotherapy kills the lymphoma cells and also the stem cells in your . You might also have radiotherapy, After the chemotherapy and radiotherapy you have the new stem cells into your bloodstream through a drip.
The cells find their way back to your bone marrow. Your body then starts making blood cells again and your bone marrow slowly recovers. The new cells also help destroy any remaining cancer cells.
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 transplant.
Research into CTCL
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 different drug treatments including:
- mogamulizumab, together with radiotherapy.
- pembrolizumab and radiotherapy
- Histone deacetylase (HDAC) inhibitors such as resminostat
Coping with skin lymphoma
Skin lymphoma and its treatment can cause physical problems. Problems might include itching or burning skin. And you might develop infections. Ask your consultant or specialist nurse for advice about how to look after your skin and ways to make it more comfortable. They will be able to suggest treatments to help with itching or burning.
It can be difficult coping with a rare type of cancer as you may not meet many people in a similar situation. Other people might notice your skin and it could make you conscious of your condition. This can affect your mood or confidence.
CTCL can affect your sex life. The lymphoma patches and rash can be uncomfortable and you might not want anyone close to you or touching you. Changes in your appearance might also affect how you feel about having sex.
Try not to feel embarrassed about discussing sexual problems with your specialist nurse or doctor. They are used to answering questions about sex and talking about these changes might be helpful. Discuss your feelings and any worries with your partner too. Your partner could go with you if you decide to have a chat with your doctor or nurse.
Other support for people with CTCL
You could also ask if there are any support groups at your hospital or within your local area. There are also charities, such as the Lymphoma Association who can provide support and information.
It might help to let friends and family know if you are finding it difficult to cope. Talking about how you feel can help. Some people decide to have counselling.
