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Cutaneous T-Cell Lymphoma

Cutaneous T-Cell Lymphoma, �

CTCL is rare 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.

How is it caused?

The causes of CTCL are unknown. CTCL, like other cancers, is not infectious and cannot be passed on to other people. CTCL is the result of an uncontrolled growth of helper T-cells. Genetic predisposition is the main cause, but other causative agents such as long-term exposure to industrial or environmental metals, organic solvents, chemical carcinogens, pesticides and herbicides are also under consideration from the research community.

What are the symptoms?

The first symptoms of skin lymphoma are dry scaly skin, a red rash and itching. The rashes are more common in areas that remain covered with clothes. Some individuals may first notice red or dark patches on the skin. These symptoms are not specific for lymphoma and most people are often first treated for more common skin conditions before lymphoma is suspected.

As the disease progresses, the red patches may become elevated � these are called plaques. Plaques may later turn into nodular or bumpy tumors. In advanced disease, ulcers can develop over these lesions. Disease may also spread to the nodes or other organs.

How is it diagnosed?

The diagnosis is made through removing of a small piece of affected skin and examining it under a microscope for abnormal cells (biopsy).

This can be done under a local anaesthetic.

What is the treatment?

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

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.

UVB therapy

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

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

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

Bexarotene (Targretin�) is a drug that is sometimes used to treat advanced CTCL. It is taken with food, as a daily capsule. Side effects of bexarotene are generally mild, and include headaches, a rash, nausea, diarrhoea and a lowered resistance to infection.

Interferon

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.

Photopheresis

This treatment is used particularly for Sezary syndrome. Photopheresis involves exposing the blood to ultraviolet light, by connecting the patient to a specialised machine.