Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is a rare type of non-Hodgkin lymphoma that affects the skin. It develops when T-cells become abnormal. T-cells are white blood cells that fight infection.

Symptoms may include:

  • raised, rash-like or itchy patches of skin
  • lumps on the skin
  • swollen lymph nodes.

Your doctor will take a small sample (biopsy) from an area of affected skin to make a diagnosis. You may also have scans to find out the stage of the lymphoma. The stage is based on how much of the skin is affected and whether it has spread elsewhere. Knowing the stage helps the doctors plan the right treatment for you.

CTCL usually develops very slowly. When it is at an early stage, doctors usually treat the skin directly. This may include using creams, radiotherapy or a light therapy called PUVA.

If the lymphoma is more widespread, treatments that work throughout the body may be used. These may include:

  • chemotherapy
  • interferon injections
  • retinoid tablets (Bexarotene)
  • light therapy for the blood called extracorporeal phototherapy (ECP).

You may be invited to join a clinical trial looking at new ways of treating CTCL. You can talk about this with your lymphoma doctor.

What is cutaneous T-cell lymphoma (CTCL)?

It is best to read this information with our general information about non-Hodgkin lymphoma (NHL). If you have any more questions, you can ask your doctor or nurse at the hospital where you are having treatment.

Cutaneous means skin. Cutaneous T-cell lymphoma (CTCL) is a type of NHL that affects the skin. It develops when T-cells (also called T-lymphocytes) become abnormal. T-cells are white blood cells that fight infection.

There are different types of CTCL. The most common are called mycosis fungoides and Sézary syndrome.

Mycosis fungoides (MF)

This is the most common type of CTCL. MF is usually a very slow-growing type of lymphoma that often only affects the skin.

For most people, MF will develop slowly or stay under control for many years. In a small number of people, MF may spread to other parts of the body over time.

Sézary syndrome

In this type of CTCL, the abnormal T-cells affect the skin but are also found in other areas of the body such as the blood and lymph nodes. Lymph nodes are part of the body’s lymphatic system. They filter out disease and germs (bacteria and viruses) as fluid passes through them.

The lymphatic system
The lymphatic system

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Causes and risk factors for CTCL

The causes of CTCL are not known. Like other cancers, it is not infectious and can't be passed on to other people. It usually affects people aged 40 to 60 and is more common in men than women.

We have more information about risk factors for lymphoma.


Signs and symptoms of CTCL

In the early stages, CTCL can look like other skin conditions, such as eczema or psoriasis. This may mean it takes some time to diagnose.

CTCL may cause changes to the skin, including:

  • red, rash-like patches
  • itchy, sometimes painful, areas
  • raised patches (plaques)
  • lumps.

Some people with CTCL have lumps where a lymph node is swollen.


Diagnosing CTCL

You usually start by seeing your GP. They can arrange for you to see a specialist at the hospital for tests and advice. This specialist is often a dermatologist (a doctor who treats skin problems) or a lymphoma doctor.

The specialist doctor will check your skin. They will take a small sample (a biopsy) from an area of affected skin. You usually have an injection to numb the area first. The sample is then examined under a microscope for abnormal cells.

The doctor will check your lymph nodes for swelling. You will also have blood tests to check for abnormal T-cells in your blood.

You may have other tests to check whether there is lymphoma in other parts of the body. These tests may include:

  • scans
  • a lymph node biopsy
  • a bone marrow sample.


Staging CTCL

The stage of CTCL describes how much of the skin is affected and if it has spread anywhere else.

CTCL usually develops very slowly. The symptoms can stay the same for many years and many people never progress beyond the first stage. A few people don’t have any symptoms until they notice raised lumps (tumours) on the skin.

Stage 1

There are red patches or raised red patches (plaques) on the skin. This stage is sometimes divided into:

• Stage 1A – less than 10% of the skin is affected.

• Stage 1B – 10% or more of the skin is affected.

Stage 2A

Skin symptoms are the same as in stage 1. Some lymph nodes are enlarged. There may be signs of abnormal lymphocytes in the lymph nodes.

Stage 2B

There may be one or more tumours on the skin that are greater than 1cm. The lymph nodes may or may not be affected.

Stage 3

More than 80% of the skin is red (erythroderma). The lymph nodes may or may not be affected.

Stage 4A

There may be any of the skin symptoms described in the previous stages. Abnormal lymphocytes have spread to the lymph nodes or the blood. 

Stage 4B

There may be any of the skin symptoms described in the previous stages. Abnormal lymphocytes have spread to the lymph nodes or the blood. The lymphoma has spread to other organs in the body, such as the liver, lungs or bone marrow.


Treating CTCL

Several treatments can be used for CTCL. Sometimes a combination of treatments is used. Your specialist will discuss your options with you.

Doctors usually use treatments to treat the skin directly when CTCL is at an early stage. These include:

  • steroid cream
  • chemotherapy cream (topical chemotherapy)
  • PUVA (light treatment)
  • radiotherapy.

Sometimes treatments that work throughout the body are used. These treatments may be given as tablets, or as an injection into a vein or under the skin. They are then carried in the blood to all areas of the body. You may have these treatments if:

  • direct skin treatments are not controlling the CTCL
  • CTCL is affecting the lymph nodes or other organs
  • there are a lot of tumours on the skin.

Treatments and pregnancy

Some treatments for CTCL can be harmful to an unborn child or a baby conceived during treatment. If you are a woman having treatment for CTCL, it is important to tell your doctor if you are pregnant or become pregnant. If you are a man, you may be advised not to father a child during treatment for CTCL. You may also need to use barrier contraception, such as a condom, if your partner is pregnant. You doctor will explain what contraception you should use and for how long after treatment.

Skin care

Skin care is important for all stages of CTCL. Your specialist nurse and doctor can give you more advice about looking after your skin. They may suggest moisturising creams or bath lotions to prevent dry skin. This helps your skin feel more comfortable, less itchy and helps prevent skin infections.

Skin creams (topical treatments)

Topical treatments are drugs that are put directly onto the area where they are needed. Your doctor may prescribe steroid or chemotherapy cream to treat areas of your skin. Your doctor, a nurse or a pharmacist will explain how to use the cream. It is important to follow the instructions they give you.

Steroids are drugs that help reduce redness and swelling. A steroid cream may be the only treatment you need to control early-stage CTCL.

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. Your doctor or nurse will explain exactly how to use a chemotherapy cream and which areas of skin to use it on. You must wear gloves to protect your hands when you use it.

Some people may have a reaction to skin creams. Tell your doctor if you have any side effects, such as redness or a burning sensation in the area.

PUVA treatment

PUVA (sometimes called photochemotherapy) is mainly used to treat skin symptoms during early CTCL. It uses a drug called psoralen (P), which makes your skin sensitive to ultraviolet light (UVA). You can take psoralen as a tablet or you can soak in a bath with psoralen liquid.

Once the drug has had time to collect in your skin, you go into an enclosed, air-conditioned cabinet to have the ultraviolet light treatment. You may have this treatment a few times a week over several weeks until your skin improves. Sometimes PUVA can be used again if your skin symptoms come back.

Side effects of PUVA

UVA treatment tans the skin and can cause early ageing of the skin. It also increases the risk of developing other types of skin cancer. The number of PUVA treatments you have is limited to reduce this risk. You should check your skin regularly after treatment for signs of skin cancer. Your doctor or specialist nurse can give you more information.

During your treatment, the drug psoralen will make you very sensitive to the sun. It is important to cover up and protect your skin from the sun to avoid sunburn. Don’t use a sunbed or sun lamp.

You need to be extra careful for 24 hours after the psoralen. During this time, you should protect your skin from sunlight even through windows. If you took psoralen as tablets, you should wear sunglasses to protect your eyes outdoors or near fluorescent lights.

Psoralen tablets can make you feel sick. Take your tablets with food. If you feel sick, your doctor can give you anti-sickness (anti-emetic) drugs to help. Sometimes they may change the psoralen to a different drug.

During your course of PUVA, your skin may become itchy or red, like mild sunburn. Tell your doctor or nurse if this happens. They can give you advice about creams that may help.

UVB therapy

UVB light (another type of ultraviolet light) is also sometimes used to treat CTCL. The treatment is given in a similar way to PUVA, using an air-conditioned cabinet containing ultraviolet lights. But you don’t need to take a drug to make your skin more sensitive to light. You may have treatment a few times a week for several weeks.

Side effects of UVB therapy

UVB therapy causes similar skin side effects to PUVA. It also increases the risk of other types of skin cancer. You should check your skin regularly after treatment for signs of skin cancer. Your doctor or specialist nurse can give you more information.

Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells. It is an effective treatment for early CTCL. It can be used to treat an area of the body affected by CTCL.

Possible side effects include redness of the skin and hair loss in the area being treated. Let your doctor or radiographer know about any side effects so they can help.

Total skin electron beam therapy (TSEBT)

TSEBT treats all the skin on the body with radiotherapy at once. It may be used:

  • if CTCL has not spread below the skin surface
  • if other direct skin treatments have not helped or have stopped working.

TSEBT can be repeated if needed. This treatment is only available at some hospitals.

The side effects are mainly skin changes and hair loss. Some sweat glands may stop working. Your doctor can give you more information about this treatment and its effects.

Chemotherapy

Chemotherapy may be given to help control CTCL. It is used if there are lots of tumours on the skin or if the lymph nodes or other organs are affected. It may also be used if other treatments are not controlling the CTCL. Chemotherapy may be given by injection into a vein or as a tablet. You may be given a single drug or a combination of drugs. Chemotherapy drugs used to treat CTCL include:

Interferon

Interferon is a protein that occurs naturally in the body. A man-made version may be used to treat CTCL. It works by boosting the body’s immune system to control the lymphoma. Interferon is injected under the skin (subcutaneously). It may be given on its own or with other treatments to control CTCL.

Bexarotene

Bexarotene (Targretin®) is a type of drug called a retinoid. It may be used to treat CTCL that has come back after other treatments. It is taken daily as capsules and may be used with other treatments, such as PUVA.

Bexarotene can affect the levels of fats in the blood and how your thyroid gland and liver are working. You will have regular blood tests to check for side effects. You doctor will explain the possible side effects and what to expect.

Extra-corporeal photopheresis (ECP)

ECP is a type of PUVA treatment for the blood. It can be used to treat abnormal lymphocytes in the blood. This may help improve any skin symptoms caused by CTCL.

You have a needle placed in each arm and these are connected to a machine. The machine takes a small amount of your blood from one arm, collects some white blood cells (which include the abnormal lymphocytes) and returns the rest of the blood to your other arm.

When enough white blood cells have been collected, a drug called psoralen is added to them. This makes the cells sensitive to ultraviolet light (UVA). The white blood cells are treated with ultraviolet light and are then returned to your body.

After ECP, the drug psoralen will make you very sensitive to the sun. For the next 24 hours, you should protect your skin from sunlight even through windows. You should also wear sunglasses to protect your eyes outdoors or near fluorescent lights.

ECP can take several hours. You usually have treatment for 2 days as an outpatient. This is repeated every few weeks for several months. ECP is only available in some hospitals. Your doctor or nurse will give you more information.

Clinical trials for CTCL

Your doctor may talk to you about clinical trials. Clinical trials for CTCL may:

  • test new treatments or new ways of giving treatments
  • record information about your condition.


Getting support

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse. You can also call our cancer support specialists free on 0808 808 00 00. The organisations below also offer information and support:

  • Bloodwise offers support and information to people affected by blood cancers, including lymphoma.
  • Changing Faces provides free help, support, and information for people with a facial disfigurement. Runs a service providing individual teaching sessions on how to use camouflage make-up.
  • Let’s Face It provides support, information, social activities and advice on camouflage make-up for people with facial disfigurement.
  • The Lymphoma Association gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. It has a national network of people with lymphoma, and local groups.