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Macmillan and Cancerbackup merged in 2008. Together we provide free, high quality information for people affected by cancer through our publications, website and phone service. Find out more| .
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This information is about a specific type of non-Hodgkin lymphoma known as cutaneous T-cell lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma (NHL).
CTCL is rare, accounting for about one in 20 of all cases of NHL. It is a cancer of the T-lymphocytes and most often occurs in people aged between 40 and 60.
Unlike other forms of non-Hodgkin lymphoma, CTCL mainly affects the skin. It is caused by the uncontrolled growth of a type of white blood cell within the skin called a T-cell.
The most common types of CTCL are mycosis fungoides and Sezary syndrome. Sezary syndrome is a specific type of CTCL in which large areas of skin or lymph glands are affected, and abnormal T-lymphocytes are also found in the blood. Mycosis fungoides is the general name given to the other types of CTCL when the blood is not affected.
The causes of CTCL are unknown. CTCL, like other cancers, is not infectious and cannot be passed on to other people.
The diagnosis is made by removing a small piece of affected skin and examining it under a microscope for abnormal cells (this is called a biopsy).
This can be done under a local anaesthetic.
The stage of CTCL indicates how the disease is affecting the skin. There are three recognisable stages of early CTCL, although the disease will not necessarily progress through all three stages.
Premycotic or pre-tumour stage Small, raised, red patches appear on the skin – commonly on the breast and buttocks, although they can appear anywhere. At this stage the disease often looks like common skin conditions such as eczema or psoriasis.
Plaque or infiltrative stage Irregularly shaped red patches (plaques) form. Any part of the body may be affected, but the buttocks, skin folds and face are particularly common places. There may be permanent hair loss from the affected areas if the plaques are left untreated.
Tumour stage Only a small proportion of people progress to this stage (most people never progress beyond the first stage). Raised lumps (tumours) appear on the skin. The tumours and the red plaques may become deep sores (ulcerate). At this stage the cancer may also have affected the lymph nodes. Rarely, it will affect major internal organs such as the liver, lungs, and spleen.
Sezary syndrome CTCL is referred to as Sezary syndrome when large areas of the skin are affected and large numbers of abnormal lymphocytes are also found in the blood. In some people there are no plaques or tumours, but the whole skin can be red, thickened, swollen, and sore (known as l’homme rouge). The skin may also be itchy and the surface may peel off.
For practical purposes, non-Hodgkin lymphomas are divided into one of two groups – low- and high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas tend to grow more quickly.
CTCL is a low-grade lymphoma and usually develops very slowly. It may be many years before it develops from one stage to the next. Most people never progress beyond the first stage. Some people do not experience the first two stages; the appearance of a tumour (stage 3) can be the first sign of their disease.
A number of treatments can be used for CTCL, either alone or in combination. Most treatments can be used for any stage of disease. The chosen treatment often depends on how much of the skin is affected.
PUVA treatment (sometimes known as photochemotherapy) is suitable if large areas of the skin are affected. It involves taking a drug called psoralen (P), which sensitises the skin to the beneficial effects of ultraviolet light A (UVA). Once the drug has had time to collect in your skin, you enter an enclosed air-conditioned cabinet that contains ultraviolet lights. Having the treatment is like sitting under a sunlamp. The treatment may be given several times a week.
Side effects The treatment causes the skin to tan and can cause premature ageing of the skin. During the time you are having treatment you need to be careful not to expose the skin to extra ultraviolet light from the sun. Exposure to ultraviolet light can increase your risk of developing skin cancer. Your doctor or specialist nurse can discuss this with you further.
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 have a different tablet prescribed.
Other possible side effects of PUVA include itching, dizziness, and headaches.
Ultraviolet light B (UVB) can help to 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, the treatment does not include the use of a drug to make the skin more sensitive. Treatment may be given several times a week.
Side effects Like PUVA treatment, UVB therapy causes the skin to tan and can cause premature ageing of the skin. Sometimes the skin may become red, like a mild sunburn. During the time you are having treatment you need to take care to avoid exposing the skin to further ultraviolet light from the sun. Exposure to ultraviolet light can increase your risk of developing skin cancer. Your doctor or specialist nurse can discuss this with you further.
Radiotherapy| is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. It may be used for early-stage disease if only one or two small areas of skin are affected by CTCL.
Radiotherapy may be used to treat one or two small areas of skin affected by CTCL. It may also be used to treat the whole skin surface if the lymphoma is more widespread but has not penetrated below the skin surface. Treatment to the whole skin surface is called total skin electron beam treatment (TSEB).
Side-effects are mainly skin changes and hair loss in the area of skin that is treated. Sweat glands in treated areas may no longer work. The extent of side-effects will depend on the size of the area treated.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. This may be applied in the form of an ointment, directly on to the whole skin surface. Your doctor or pharmacist will tell you how to do this. It is vital to follow instructions carefully, and to only put the cream where you are told to.
If the condition is getting worse (progressing) and not responding to other forms of treatment, chemotherapy by injection into a vein may stop or slow it. This type of chemotherapy treatment, treating the whole body (systemic therapy), is usually only used for more advanced CTCL.
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.
Bexarotene (Targretin®) belongs to a group of drugs called retinoids. It's taken daily as a capsule. Bexarotene is sometimes used to treat CTCL that has come back after other treatments. It can be given along with treatments such as PUVA.
This is a type of biological therapy. It contains a toxin that binds to the cancerous T-cells and destroys them. Denileukin is a newer drug that can be used to treat CTCL which has come back after other treatments.
This drug is a monoclonal antibody. Monoclonal antibodies recognise and attach onto 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| can be given when CTCL has come back after other treatments.
This treatment is used particularly for Sezary syndrome. Photopheresis involves exposing the blood to ultraviolet light, by connecting the patient to a specialised machine. Our cancer support specialists| can tell you more about this type of treatment.
New treatments for CTCL are being researched all the time. The use of interferon alongside existing treatments is currently being researched.
Retinoids are being used in some research trials|. These are tablets which increase the production of normal lymphocytes and can be used to improve the effectiveness of PUVA treatment.
Your doctor may invite you to take part in a clinical trial to compare a new treatment against the best available standard treatment. Your doctor must discuss the treatment with you, and have your informed consent before entering you into any clinical trial.
Skin moisturising creams can reduce skin discomfort. Other preparations can be added to bath water to keep the skin supple and relieve irritation.
Your doctor may prescribe steroid creams or ointments that can help the skin to 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 |for more information and support.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography|.
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