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Cutaneous T-cell lymphoma


Table of Contents

OVERVIEW OF PDQ
What is PDQ?
How to use PDQ
DESCRIPTION
What is cutaneous T-cell lymphoma?
STAGE EXPLANATION
Stages of cutaneous T-cell lymphoma
Stage I
Stage II
Stage III
Stage IV
Recurrent
TREATMENT OPTION OVERVIEW
How cutaneous T-cell lymphoma is treated
Treatment by stage
STAGE I CUTANEOUS T-CELL LYMPHOMA
STAGE II CUTANEOUS T-CELL LYMPHOMA
STAGE III CUTANEOUS T-CELL LYMPHOMA
STAGE IV CUTANEOUS T-CELL LYMPHOMA
RECURRENT CUTANEOUS T-CELL LYMPHOMA
TO LEARN MORE

OVERVIEW OF PDQ


What is PDQ?

PDQ is a computer system that gives up-to-date information on cancer and its prevention, detection, treatment, and supportive care. It is a service of the National Cancer Institute (NCI) for people with cancer and their families and for doctors, nurses, and other health care professionals.

To ensure that it remains current, the information in PDQ is reviewed and updated each month by experts in the fields of cancer treatment, prevention, screening, and supportive care. PDQ also provides information about research on new treatments (clinical trials), doctors who treat cancer, and hospitals with cancer programs. The treatment information in this summary is based on information in the PDQ summary for health professionals on this cancer.


How to use PDQ

PDQ can be used to learn more about current treatment of different kinds of cancer. You may find it helpful to discuss this information with your doctor, who knows you and has the facts about your disease. PDQ can also provide the names of additional health care professionals who specialize in treating patients with cancer.

Before you start treatment, you also may want to think about taking part in a clinical trial. PDQ can be used to learn more about these trials. A clinical trial is a research study that attempts to improve current treatments or finds information on new treatments for patients with cancer. Clinical trials are based on past studies and information discovered in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help patients with cancer. Information is collected about new treatments, their risks, and how well they do or do not work. When clinical trials show that a new treatment is better than the treatment currently used as "standard" treatment, the new treatment may become the standard treatment. Listings of current clinical trials are available on PDQ. Many cancer doctors who take part in clinical trials are listed in PDQ.

To learn more about cancer and how it is treated, or to learn more about clinical trials for your kind of cancer, call the National Cancer Institute's Cancer Information Service. The number is 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. The call is free and a trained information specialist will be available to answer cancer-related questions.

PDQ is updated whenever there is new information. Check with the Cancer Information Service to be sure that you have the most up-to-date information.


DESCRIPTION


What is cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma is a disease in which certain cells of the lymph system (called T-lymphocytes) become cancer (malignant) and affect the skin. Lymphocytes are infection-fighting white blood cells that are made in the bone marrow and by other organs of the lymph system. T-cells are special lymphocytes that help the body's immune system kill bacteria and other harmful things in the body.

The lymph system is part of the immune system and is made up of thin tubes that branch, like blood vessels, into all parts of the body, including the skin. Lymph vessels carry lymph, a colorless, watery fluid that contains lymphocytes. Along the network of vessels are groups of small, bean-shaped organs called lymph nodes. Clusters of lymph nodes are found in the underarm, pelvis, neck, and abdomen. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood), the thymus (a small organ beneath the breastbone), and the tonsils (an organ in the throat) are also part of the lymph system.

There are several types of lymphoma. The most common types of lymphoma are called Hodgkin's disease and non-Hodgkin's lymphoma. These types of lymphoma usually start in the lymph nodes and the spleen. See the patient information summaries on adult or childhood non-Hodgkin's lymphoma or adult or childhood Hodgkin's disease for treatment of these cancers.

Cutaneous T-cell lymphoma usually develops slowly over many years. In the early stages, the skin may itch, and dry, dark patches may develop on the skin. As the disease gets worse, tumors may form on the skin, a condition called mycosis fungoides. As more and more of the skin becomes involved, the skin may become infected. The disease can spread to lymph nodes or to other organs in the body, such as the spleen, lungs, or liver. When large numbers of the tumor cells are found in the blood, the condition is called the Sezary syndrome.

If there are symptoms of cutaneous lymphoma, a doctor may remove a growth from the skin and look at it under a microscope. This is called a biopsy.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in the skin or has spread to other places in the body) and the patient's general state of health.

There are several other types of cancer that start in the skin. The most common are basal cell cancer and squamous cell cancer, which are covered in the PDQ patient information summary on skin cancer. Another type of skin cancer called melanoma is covered in the patient information summary on melanoma. Kaposi's sarcoma, a rare type of cancer that occurs most commonly in patients with the Acquired Immunodeficiency Syndrome (AIDS), also affects the skin. See the PDQ patient information summary on Kaposi's sarcoma for treatment of this cancer. Cancers that start in other parts of the body may also spread (metastasize) to the skin.


STAGE EXPLANATION


Stages of cutaneous T-cell lymphoma

Once cutaneous T-cell lymphoma is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. A doctor needs to know the stage of the disease to plan treatment. The following stages are used for cutaneous T-cell lymphoma:


Stage I

The cancer only affects parts of the skin, which has red, dry, scaly patches, but no tumors. The lymph nodes are not larger than normal.


Stage II

Either of the following may be true:

The skin has red, dry, scaly patches, but no tumors. Lymph nodes are larger
than normal, but do not contain cancer cells.

There are tumors on the skin. The lymph nodes are either normal or are
larger than normal, but do not contain cancer cells.


Stage III

Nearly all of the skin is red, dry, and scaly. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells.


Stage IV

The skin is involved, in addition to either of the following:

Cancer cells are found in the lymph nodes.

Cancer has spread to other organs, such as the liver or lung.


Recurrent

Recurrent disease means that the cancer has come back after it has been treated. It may come back where it started or in another part of the body.


TREATMENT OPTION OVERVIEW


How cutaneous T-cell lymphoma is treated

There are treatments for all patients with cutaneous T-cell lymphoma. Three kinds of treatment are commonly used:

Biological therapy (using the body's immune system to fight cancer) is being tested in clinical trials.

Radiation therapy uses high-energy rays to kill cancer cells and shrink tumors. In cutaneous T-cell lymphoma, special rays of tiny particles called electrons are commonly used to treat all of the skin. This is called total skin electron beam radiation therapy, or TSEB radiation therapy. Electron beam radiation may also be given to smaller areas of the skin. This kind of radiation only goes into the outer layers of the skin. Another type of radiation uses x-rays to kill cancer cells. The x-rays are usually directed to only certain areas of the body, but there are studies using x-rays directed at the whole body (total body irradiation).

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy given in this way is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body. In cutaneous T-cell lymphoma, chemotherapy drugs may be given in a cream or lotion put on the skin. This is called topical chemotherapy.

Phototherapy uses light to kill cancer cells. A drug that makes cancer cells sensitive to light is given to the patient and then a special light is used to shine on the cancer cells to kill them. In one type of phototherapy, called PUVA therapy, a patient will receive a drug called psoralen, and then ultraviolet A light will be shone on the skin. In another type of phototherapy, called extracorporeal photochemotherapy, the patient will be given drugs, and then some of the blood cells will be taken from the body, put under a special light, and put back into the body. If phototherapy is given, directions from the doctor should be followed as to the amount of sunlight the patient should receive.

Biological therapy tries to get the body to fight cancer. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body's natural defenses against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy.

Bone marrow transplantation is used to replace the bone marrow with healthy bone marrow. First, all of the bone marrow in the body is destroyed with high doses of chemotherapy with or without radiation therapy. Healthy marrow is then taken from another person (a donor) whose tissue is the same as or almost the same as the patient's. The donor may be a twin (the best match), a brother or sister, or another person not related. The healthy marrow from the donor is given to the patient through a needle in the vein, and the marrow replaces the marrow that was destroyed. A bone marrow transplant using marrow from a relative or unrelated person is called an allogeneic bone marrow transplant.

Another type of bone marrow transplant, called autologous bone marrow transplant, is being studied in clinical trials. To do this type of transplant, bone marrow is taken from the patient and treated with drugs to kill any cancer cells. The marrow is then frozen to save it. Next, the patient is given high-dose chemotherapy with or without radiation therapy to destroy all of the remaining marrow. The frozen marrow that was saved is then thawed and given back to the patient through a needle in a vein to replace the marrow that was destroyed.

Another type of autologous transplant is called a peripheral blood stem cell transplant. The patient's blood is passed through a machine that removes the stem cells (immature cells from which all blood cells develop), then returns the blood back to the patient. This procedure is called leukapheresis and usually takes 3 or 4 hours to complete. The stem cells are treated with drugs to kill any cancer cells and then frozen until they are transplanted back to the patient. This procedure may be done alone or with an autologous bone marrow transplant.

A greater chance for recovery occurs if the doctor chooses a hospital which does more than 5 bone marrow transplantations per year.


Treatment by stage

Treatment of cutaneous T-cell lymphoma depends on the stage of the disease, and the patient's age and overall health.

Standard treatment may be considered because of its effectiveness in patients in past studies, or participation in a clinical trial may be considered. Most patients with cutaneous T-cell lymphoma are not cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials are ongoing in many parts of the country for most stages of cutaneous T-cell lymphoma. To learn more about clinical trials, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.


STAGE I CUTANEOUS T-CELL LYMPHOMA

Treatment may be one of the following:

1. Phototherapy (PUVA therapy) with or without biological therapy.

2. Total skin electron beam radiation therapy (TSEB radiation therapy).

3. Topical chemotherapy.

4. Local electron beam or x-ray therapy to reduce the size of the tumor or to relieve symptoms.

5. Clinical trials of phototherapy.

6. Interferon alfa (biological therapy) alone or in combination with topical therapy.


STAGE II CUTANEOUS T-CELL LYMPHOMA

Treatment may be one of the following:

1. Phototherapy (PUVA therapy) with or without biological therapy.

2. Total skin electron beam radiation therapy (TSEB radiation therapy).

3. Topical chemotherapy.

4. Local electron beam or x-ray therapy.

5. Interferon alfa (biological therapy) alone or in combination with topical therapy.


STAGE III CUTANEOUS T-CELL LYMPHOMA

Treatment may be one of the following:

1. Phototherapy (PUVA therapy) with or without biological therapy.

2. Total skin electron beam radiation therapy (TSEB radiation therapy).

3. Topical chemotherapy.

4. Local electron beam or x-ray therapy.

5. Systemic chemotherapy with or without therapy to the skin.

6. Chemotherapy for mycosis fungoides and Sezary syndrome.

7. Extracorporeal photochemotherapy.

8. Interferon alfa (biological therapy) alone or in combination with topical therapy.

9. Retinoids.


STAGE IV CUTANEOUS T-CELL LYMPHOMA

Treatment may be one of the following:

1. Systemic chemotherapy.

2. Topical chemotherapy.

3. Total skin electron beam radiation therapy (TSEB radiation therapy).

4. Phototherapy (PUVA therapy) with or without biological therapy.

5. Local electron beam or x-ray therapy.

6. Chemotherapy for mycosis fungoides and Sezary syndrome.

7. Extracorporeal photochemotherapy

8. Interferon alfa (biological therapy) alone or in combination with topical therapy.

9. Monoclonal antibody therapy.

10. Retinoids.


RECURRENT CUTANEOUS T-CELL LYMPHOMA

Treatment depends on many factors, including the type of treatment the patient received before. Depending on the patient's condition, treatment may be one of the following:

1. Local electron beam or x-ray therapy.

2. Total skin electron beam radiation therapy (TSEB radiation therapy).

3. Phototherapy (PUVA therapy).

4. Topical chemotherapy.

5. Systemic chemotherapy.

6. Extracorporeal photochemotherapy.

7. Clinical trials of biological therapy.

8. Clinical trials of bone marrow transplantation.


TO LEARN MORE

TO LEARN MORE..... CALL 1-800-4-CANCER

To learn more about cutaneous T-cell lymphoma, call the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. By dialing this toll-free number, trained information specialists can answer your questions.

The Cancer Information Service also has booklets about cancer that are available to the public on request. The following general booklets on questions related to cancer may be helpful:

What You Need To Know About Cancer
Taking Time: Support for People with Cancer and the People Who Care About
Them
What Are Clinical Trials All About?
Chemotherapy and You: A Guide to Self-Help During Treatment
Radiation Therapy and You: A Guide to Self-Help During Treatment
Eating Hints for Cancer Patients
Advanced Cancer: Living Each Day
When Cancer Recurs: Meeting the Challenge Again

There are many other places where people can get material and information about cancer treatment and services. The social service office at a hospital can be checked for local and national agencies that can help with getting information about finances, getting to and from treatment, getting care at home, and dealing with problems.

For more information from the National Cancer Institute, please write to this address:

National Cancer Institute
Office of Cancer Communications
31 Center Drive, MSC 2580
Bethesda, MD 20892-2580

Date Last Modified: 03/1998


If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.


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