National Cancer Institute


Posted Date: Jun 6, 2003

TABLE OF CONTENTS


Description

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What is mycosis fungoides and the Sézary syndrome?

Mycosis fungoides and the Sézary syndrome 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 lymphomas are called Hodgkin's lymphomas and non-Hodgkin's lymphomas. These types of lymphoma usually start in the lymph nodes and the spleen. (Refer to the PDQ® summaries on Adult Non-Hodgkin's Lymphoma Treatment; Childhood Non-Hodgkin's Lymphoma Treatment; Adult Hodgkin's Lymphoma Treatment; and Childhood Hodgkin's Disease Treatment for more information.)

Mycosis fungoides and the Sézary syndrome 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 Sézary 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 (refer to the PDQ® summary on Skin Cancer Treatment for more information). Another type of skin cancer called melanoma (refer to the PDQ® summary on Melanoma Treatment for more information). Kaposi's sarcoma, a rare type of cancer that occurs most commonly in patients with the Acquired Immunodeficiency Syndrome (AIDS), also affects the skin (refer to the PDQ® summary on Kaposi's Sarcoma Treatment for more information). Cancers that start in other parts of the body may also spread (metastasize) to the skin.


Stage Explanation

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Stages of mycosis fungoides and the Sézary syndrome

Once mycosis fungoides and the Sézary syndrome 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 mycosis fungoides and the Sézary syndrome:


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:

  1. The skin has red, dry, scaly patches, but no tumors. Lymph nodes are larger than normal, but do not contain cancer cells.
  2. 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:

  1. Cancer cells are found in the lymph nodes.
  2. 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 Options Overview

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How mycosis fungoides and the Sézary syndrome are treated

There are treatments for all patients with mycosis fungoides and the Sézary syndrome. Three kinds of treatment are commonly used:

  • Radiation therapy (using high-energy rays to kill cancer cells).
  • Chemotherapy (using drugs to kill cancer cells).
  • Phototherapy (using light plus special drugs to make the cancer cells more sensitive to the light).

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 Mycosis Fungoides/Sézary Syndrome

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


Stage II Mycosis Fungoides/Sézary Syndrome

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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.
  6. Retinoids.


Stage III Mycosis Fungoides/Sézary Syndrome

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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. Local electron beam or x-ray therapy.
  4. Chemotherapy for mycosis fungoides and Sézary syndrome.
  5. Interferon alfa (biological therapy) alone or in combination with topical therapy.
  6. Biological therapy.
  7. Systemic chemotherapy with or without therapy to the skin.
  8. Extracorporeal photochemotherapy.
  9. Topical chemotherapy.
  10. Retinoids.


Stage IV Mycosis Fungoides/Sézary Syndrome

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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. Local electron beam or x-ray therapy.
  4. Chemotherapy for mycosis fungoides and Sézary syndrome.
  5. Interferon alfa (biological therapy) alone or in combination with topical therapy.
  6. Biological therapy.
  7. Systemic chemotherapy with or without therapy to the skin.
  8. Topical chemotherapy.
  9. Extracorporeal photochemotherapy.
  10. Monoclonal antibody therapy.
  11. Retinoids.


Recurrent Mycosis Fungoides/Sézary Syndrome

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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.


Changes to This Summary (06/06/2003)

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The PDQ® cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.


To Learn More

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Call

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Web sites and Organizations

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

LiveHelp

The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write

For more information from the NCI, please write to this address:

  • NCI Public Inquiries Office
  • Suite 3036A
  • 6116 Executive Boulevard, MSC8322
  • Bethesda, MD 20892-8322


About PDQ®

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PDQ® is a comprehensive cancer database available on NCI's Web site.

PDQ® is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ® is available online at NCI's Web site. PDQ® is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ® contains cancer information summaries.

The PDQ® database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ® cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ® also contains information on clinical trials.

Before starting treatment, patients may want to think about taking part in a clinical trial. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ® and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ®. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.


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