Skip the secondary navigation if you do not want to read it as the next section.
Run the Cancerbackup 10K to help us reach more people with NHL. |
Skip the main content if you do not want to read it as the next section.
Skip the location trail if you do not want to read it as the next section.
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 1 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 through removing of a small piece of skin from an affected area, and examining the cells under a microscope (biopsy). This operation may be done under 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, as 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 also 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 with 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.
If necessary, radiotherapy may be given to areas of skin affected by plaques and tumours. Two or three doses of low-dose treatment from a radiotherapy machine may be given to the affected area. This form of treatment works well for CTCL.
Radiotherapy may also be used to treat the whole skin surface if the lymphoma is more widespread, but has not penetrated below the skin surface. This treatment is called total skin electron beam treatment. It is only given once and then may be followed up with further PUVA treatments if necessary. Possible side effects include loss of hair and nails, and the sweat glands in treated areas may no longer work.
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.
Bexarotene (Targretin®) is a drug that is sometimes used to treat advanced CTCL. It is taken as a capsule daily with food. Side effects of bexarotene (pronounced bex-air-oh-teen) are generally mild, and include headaches, a rash, a lowered resistance to infection, nausea, and diarrhoea.
Interferon (in-ter-fear-ron) 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.
This treatment is used particularly for Sezary syndrome. Photopheresis (photo-fer-e-sis) involves exposing the blood to ultraviolet light, by connecting the patient to a specialised machine. Our nurses 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.
The need for practical and emotional support will of course be individual. For some people with CTCL, life may seem largely unaffected; for others the diagnosis of cancer may be a cause of great fear and distress. If you would like to discuss the condition, its treatment, or the practical and emotional problems of living with CTCL, please contact our cancer support service.
This factsheet has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
Over 27,000 people visit our online NHL section every month – help us keep this information up to date >> |
Cancerbackup is a national charity and we rely on voluntary donations. To donate, please call 020 7696 9003 and tell us you have visited our website. Thank you.
