Cutaneous T-cell lymphoma (CTCL)
Cutaneous T-cell lymphoma is a type of non-Hodgkin lymphoma of the skin (cutaneous means skin).
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.
Non-Hodgkin lymphoma is a cancer of the lymphatic system. This is part of the body’s immune system and helps us fight infection.
It's made up of organs such as the bone marrow, thymus, spleen and the lymph nodes (or lymph glands). Lymph nodes are connected by a network of tiny lymphatic vessels that contain lymph fluid.
There is also lymphatic tissue in other organs, such as the skin, lungs and stomach.
There are lymph nodes all over the body. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything harmful or anything that the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.
Lymph fluid contains lymphocytes. These are a type of white blood cell that help the body fight infection and disease.
Lymphocytes start to grow in the bone marrow, where blood cells are made. The two main types of lymphocyte are B-cells and T-cells.
B-cells mature in the bone marrow, while T-cells mature in the thymus gland behind the breast bone. When they're mature, both B-cells and T-cells help fight infections.
Lymphoma is a disease in which either T-cells or B-cells grow in an uncontrolled way.
There are many different types of non-Hodgkin lymphomas. They're grouped (or classified) according to certain characteristics, such as the type of cell involved (B-cells or T-cells) and what it looks like under a microscope. The most widely used classification system is produced by the World Health Organisation.
Cutaneous T-cell lymphoma (CTCL)
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CTCL is rare and usually affects people aged 40-60. It's caused by T-cells in the skin growing in an uncontrolled way. CTCL is more common in men than in women.
Mycosis fungoides (MF)
This is the most common type of CTCL. It is a very slow growing form of lymphoma and usually only affects the skin. Treatments can often keep it under control for many years.
There are different stages of MF, but, it doesn't have to progress from one to the other. Over time, in a small number of people, it may spread to other parts of the body.
This is an advanced type of CTCL. Large areas of the skin are affected with redness (erythroderma) and scaling. The skin can be very itchy and may look thicker in parts. The lymph nodes are swollen and there are abnormal lymphocytes in the blood.
The causes of CTCL are unknown. Like other cancers, it’s not infectious and can't be passed on to other people.
The symptoms of CTCL will depend on the stage it is at when you are diagnosed. In the very early stage, CTCL can look like skin conditions such as eczema or psoriasis.
There are red, rash-like patches on the skin. The skin may be scaly and the patches are sometimes raised (plaques). Itching is a common symptom.
There may be one or more lumps (tumours) on the skin and some people may have swollen lymph nodes.
In the advanced stage, large areas of skin are affected. The skin is very red, scaly and itchy, and it may be painful. It's often swollen and looks thicker in parts.
The diagnosis is made by removing a small piece of affected skin (skin biopsy) and examining it under a microscope for abnormal cells. You can have this done under local anaesthetic.
Your doctor will also carry out a full examination of your skin and lymph nodes. You will have blood tests taken to check if there are any abnormal lymphocytes present.
Some people may also have scans such as a CT (computerised tomography) scan or a MRI (magnetic resonance imaging) scan to check that the lymphoma hasn't spread.
If your lymph nodes are swollen, you may have a biopsy or have them removed so they can be examined under a microscope for lymphoma cells.
Occasionally a sample of the bone marrow may be taken (bone marrow biopsy) and examined under the microscope.
Our general information about non-Hodgkin lymphoma explains these tests in more detail.
The stage of CTCL describes how much of the skin is affected and whether it has spread anywhere else.
CTCL usually develops very slowly. The symptoms can remain the same for many years and most people never progress beyond the first stage.
Occasionally, some people don't experience the early stages of CTCL and their first symptoms may be tumours (raised lumps) on the skin.
There are red patches and/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 surface is affected.
Skin symptoms are the same as in stage 1. Some lymph nodes are enlarged, but the lymphoma cells have not spread there.
There may be one or more tumours on the skin.
More than 80% of the skin is red (erythroderma).
There may be any of the skin symptoms described in the previous stages. There are lymphoma cells in the lymph nodes or high numbers of lymphoma cells in the blood, or both.
There may be any of the skin symptoms described in the previous stages. Lymphoma cells may or may not have spread to the lymph nodes and/or blood. The lymphoma has spread to other organs in the body such as the liver.
The treatment you have will depend on the stage of the CTCL. Different treatments can be used and you may have a combination of them. Your specialist will discuss the treatment options with you.
In early CTCL, treatments given directly to the skin are usually used. This includes steroid cream, chemotherapy cream (topical chemotherapy), PUVA or radiotherapy. These are explained below.
If CTCL is advanced or isn't responding to other treatments, systemic treatments are used. Chemotherapy (see below) or drugs called biological therapies are given directly into your bloodstream and can treat the whole body (systemic). You may also be given treatments directly to the skin.
Creams to the skin (topical treatments)
Steroid cream applied to the skin may be the only treatment needed for early stage CTCL.
Chemotherapy creams may be used. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is also given as a cream that can be applied to the affected skin.
You'll need to make sure you only put the cream on the area where you've been told to apply it and you must wear gloves to protect your hands.
Your doctor, a nurse or a pharmacist will explain this to you. It's important to follow the instructions they give you carefully. Some people may have a reaction to the cream. Let your doctor know if you have any side effects, such as redness or a burning sensation in the area.
PUVA (sometimes known as photochemotherapy) is an effective treatment for early CTCL, especially for treating plaques (raised red patches). It involves taking a drug called psoralen (P), which makes your skin sensitive to ultraviolet light (UVA).
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 done a few times a week, with treatments reducing as skin symptoms improve. Treatment with PUVA is sometimes continued when the symptoms have gone away. This is done to help keep CTCL in remission (when there are no signs of the disease).
Side effects of PUVA
This treatment tans the skin and can cause early ageing of the skin. Sometimes the skin may become red, like mild sunburn. Over the period of your treatment, you'll need to be careful not to expose your skin to extra ultraviolet light from the sun. Exposure to ultraviolet light can increase the risk of developing other types of skin cancer. The number of treatments you have with PUVA is limited to reduce the risk of this happening, and your skin is carefully monitored. Your doctor or specialist nurse can discuss this with you further. We have more information on taking care of your skin in the sun.
Psoralen makes the eyes more sensitive to ultraviolet light, so you will need to wear protective glasses for around 12 hours from the time the drug is taken. The psoralen tablets can make you feel sick. If this happens you can be given anti-sickness (anti-emetic) drugs or it may be possible to be prescribed a different tablet.
Other possible side effects of PUVA include itching, dizziness and headaches.
UVB light (ultraviolet light from wavelength B) can help slow down the growth of skin cells and may be used to treat CTCL. The treatment is delivered in a similar way to PUVA, using an air-conditioned cabinet containing ultraviolet lights. However, you don’t need to take a drug to make your skin more sensitive to light. Treatment may be given a few times a week.
Side effects of UVB
UVB therapy causes similar skin side effects to PUVA.
Because ultraviolet light increases the risk of other types of skin cancer, you will also need to avoid exposing the skin to extra UV light from the sun. Your doctor or specialist nurse can discuss this with you further.
Radiotherapy is the use of high-energy rays to destroy cancer cells. It's an effective treatment for early CTCL, when there are small areas of affected skin in the one area. It can also be used to ease skin symptoms when CTCL is advanced. The side effects are redness of the skin and hair loss in the area being treated.
Total electron beam therapy (EBT)
This is a type of radiotherapy. It can be used to treat larger areas of CTCL, that haven’t spread below the skin surface. The treatment can be repeated if necessary. It's only available at some specialist centres.
The side effects are mainly skin changes and hair loss in the area of skin that's being treated. Sweat glands in treated areas may no longer work. The extent of side effects will depend on the size of the area treated.
If CTCL is advanced or is not responding to other treatments, chemotherapy by injection into a vein, or as a tablet, may be given to help control it. Low doses of a drug called methotrexate or another drug called chlorambucil can be given as tablets to treat early disease.
In advanced disease you may be given a single drug or sometimes a combination of drugs, usually by injection into a vein. Chemotherapy drugs that may be used are gemcitabine, pentostatin or liposomal doxorubicin.
Interferon is a protein that occurs naturally in the body. It is sometimes injected just under the skin (subcutaneously) to boost the body’s own immune system to control the lymphoma. It may be given on its own or with other treatments, such as PUVA or bexarotene. The most common side effects of interferon are tiredness, aches and pains, fever and a low mood.
Bexarotene (Targretin®) belongs to a group of drugs called retinoids. It can be taken daily as a capsule to treat CTCL that has come back after other treatments have been tried. Bexarotene can be given along with other treatments such as PUVA.
Extra-corporeal photopheresis (ECP)
ECP is only used in advanced disease when the majority of the skin is affected and very red, and there are lymphoma cells in the blood. This treatment is only available in some specialist centres. ECP is a systemic (treats the whole body) type of PUVA treatment.
Blood is removed from your body by connecting you to a special machine. The blood is then treated with ultraviolet light and drugs that become active when exposed to light. The treated blood is then returned to the body. Your doctor or nurse will give you more information about this treatment.
This drug is a monoclonal antibody. Monoclonal antibodies recognise and stick to certain proteins on the surface of some cancer cells. This can trigger the immune system to attack the cancer cells and cause them to destroy themselves. Alemtuzumab, usually called Campath, is generally given when CTCL has come back after other treatments have already been tried.
New treatments for CTCL are being researched all the time. The use of interferon alongside existing treatments is currently being researched.
Your doctor must discuss the treatment with you, and have your informed consent before entering you into a trial.
Before any trial is allowed to take place, it must be approved by a research ethics committee, which protects the interests of those taking part.
You may decide not to take part or to withdraw from the trial at any stage.
You will then receive the best standard treatment available.
Using moisturising creams regularly can help look after your skin and ease some of the symptoms. Other preparations can be added to bath water to keep your skin supple and relieve irritation. Your specialist nurse and doctor can give you more advice on looking after your skin.
Your doctor may prescribe steroid creams or ointments that can help the skin heal. Follow your doctor or pharmacist’s advice, and use them sparingly.
Everyone has their own way of dealing with their illness and the different emotions they experience. Some people find it helpful to talk things over with family and friends, or their doctor or nurse. You can also contact our cancer support specialists or the organisations listed below for more information and support.
Other useful organisations
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Having a visible skin condition that affects your appearance can be very distressing. The organisations listed here can offer help and support:
British Red Cross
The British Red Cross offers a special service providing camouflage makeup and individual teaching sessions.
Changing Faces provides free help, support, and information for people with a facial disfigurement.
Let’s Face It
Let's Face It provides support, information, social activities and advice on camouflage makeup for people with facial disfigurement.
Leukaemia CARE is a national group promoting the welfare of people with leukaemia and other blood disorders, including non-Hodgkin lymphoma. Has regional support groups in many counties.
The Lymphoma Association
The Lymphoma Association gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. Has a national network of people with lymphoma and has local groups.
This section has been compiled using information from a number of reliable sources, including:
Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow up. Annals of Oncology. 2010. 21 (5): 177-180.
Primary cutaneous lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th edition. New York, NY: Springer, 2010, pp 614-5.
Guidelines for the Management of Primary Cutaneous T-cell Lymphoma. British Journal of Dermatology. July 2003. Vol 149: 1095-1107.
Lee, et al. Wintrobe’s Clinical Haematology. 12th edition. 2009. Lippincott Williams and Wilkins.
Thank you to Professor Rajnish Gupta, Consultant Medical Oncologist, and all of the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.