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Table of Contents General Information About Mycosis Fungoides and the Sézary
Syndrome
Stages of Mycosis Fungoides and the Sézary Syndrome
Recurrent Mycosis Fungoides and the Sézary Syndrome
Treatment Option Overview
Treatment Options by Stage
Treatment Options for Recurrent Mycosis Fungoides and the Sézary
Syndrome
To Learn More About Mycosis Fungoides and the Sézary Syndrome
Get More Information From NCI
Changes to This Summary (05/06/2008)
About PDQ
General Information About Mycosis Fungoides and the Sézary
Syndrome
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Mycosis fungoides and the Sézary syndrome are diseases in which
lymphocytes (a type of white blood cell) become malignant
(cancerous) and affect the skin.
Normally, the bone marrow makes blood stem cells (immature cells) that develop into mature blood stem cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. The myeloid stem cell develops into a red blood cell, white blood cell, or platelet. The lymphoid stem cell develops into a lymphoblast and then into one of three types of lymphocytes (white blood cells):
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In mycosis fungoides, T-cell lymphocytes become cancerous and affect the skin. In the Sézary syndrome, cancerous T-cell lymphocytes affect the skin and the peripheral blood.
Mycosis fungoides and the Sézary syndrome are types of cutaneous T-cell lymphoma.
This summary describes the two most common types of cutaneous T-cell lymphomas: mycosis fungoides and the Sézary syndrome. For information about other types of skin cancer or non-Hodgkin lymphoma, see the following PDQ summaries:
A possible sign of mycosis fungoides and the Sézary syndrome is a red rash on the skin.
Mycosis fungoides and the Sézary syndrome may move through the following phases:
- Premycotic phase: A scaly, red rash in areas of the body that usually are not exposed to the sun. This rash does not cause symptoms and may last for months or years. It is hard to diagnose the rash as mycosis fungoides during this phase.
- Patch phase: Thin, reddened, eczema-like rash.
- Plaque phase: Thickened, red patches or reddened skin.
- Tumor phase: Tumors form on the skin. These tumors may develop ulcers and the skin may get infected.
Sézary syndrome is an advanced form of mycosis fungoides.
In the Sézary syndrome, skin all over the body is reddened, itchy, peeling, and painful. There may also be patches, plaques, or tumors on the skin. Cancerous T-cells are found in the blood. Mycosis fungoides does not always progress to the Sézary syndrome.
Tests that examine the skin and blood are used to detect (find)
and diagnose mycosis fungoides and the Sézary syndrome.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps, the number and type of skin lesions, or anything else that seems unusual. Pictures of the skin and a history of the patient’s health habits and past illnesses and treatments will also be taken.
- Complete blood count with differential: A procedure in which a sample of blood is drawn and checked for the following:
| Enlarge | | | | Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions. |
- Peripheral blood smear: A procedure in which a sample of blood is viewed under a microscope to count different circulating blood cells (red blood cells, white blood cells, platelets, etc.) and see whether the cells look normal.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The doctor may remove a growth from the skin, which will be examined by a pathologist. More than one skin biopsy may be needed to diagnose mycosis fungoides.
- Immunophenotyping: A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process may include special staining of the blood cells. It is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.
- Immunogenotyping: A procedure in which a sample of DNA from a skin biopsy is studied to see if the genes for certain kinds of immune system proteins, such as the T-cell receptor or antibody proteins, are arranged in one pattern. Normally T-cell receptor genes and antibody genes are arranged in many different patterns. In mycosis fungoides and the Sézary syndrome, the genes are arranged in a single pattern.
Certain factors affect prognosis (chance
of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The stage of the cancer (the amount of skin affected and whether cancer has spread to the lymph
nodes, the blood, or other places in the body).
- The type of lesion (patches, plaques, or tumors).
- The number of cutaneous T-cell lymphocytes in the blood.
Mycosis fungoides and the Sézary syndrome are difficult to cure. Treatment is usually palliative, to relieve symptoms and improve the quality of life. Patients can live many years with this disease.
Back to Top Stages of Mycosis Fungoides and the Sézary Syndrome
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After mycosis fungoides and the Sézary syndrome have been
diagnosed, tests are done to find out if cancer cells have spread from the skin to other parts of the body.
The process used to find out if cancer has spread from the skin to other parts
of the body is called staging. The
information gathered from the staging process determines the stage of the disease. It is
important to know the stage in order to plan treatment. The following
procedures may be used in the staging process:
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Lymph node biopsy: The
removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
The following stages are used for mycosis fungoides and the
Sézary syndrome:
Stage I
Stage I is
divided into stage IA and stage IB as follows:
- Stage IA: Less than 10% of the skin surface is covered with patches and/or plaques.
- Stage IB: Ten percent or more of the skin surface is
covered with patches and/or plaques.
Stage II
Stage II is divided into stage IIA and stage IIB as follows:
Stage III
In stage III, nearly all of the skin is reddened and may have patches, plaques, or tumors. Lymph nodes may be enlarged but cancer has not spread to them.
Stage IV
Stage IV is divided into stage IVA and stage IVB as follows:
- Stage IVA: Most of the skin is reddened and any amount of the skin surface is covered with patches, plaques, or tumors. Cancer has spread to lymph nodes, and the lymph nodes may be enlarged.
- Stage IVB: Most of the skin is reddened and any amount of the skin surface is covered with patches, plaques, or tumors. Cancer has spread to other organs in the body. Lymph nodes may be enlarged and cancer may have spread to them.
Stages of mycosis fungoides and the Sézary syndrome may also have a B classification.
The B classification is based on how many abnormal lymphocytes are found in the blood.
Back to Top Recurrent Mycosis Fungoides and the Sézary Syndrome
Recurrent mycosis fungoides and the Sézary syndrome are cancers that have recurred (come back)
after they have been treated. The cancer may come back in the skin or in other parts of the body.
Back to Top Treatment Option Overview
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There are different types of treatment for patients with
mycosis fungoides and the Sézary syndrome cancer.
Different types of treatment are available for patients with mycosis fungoides and the Sézary syndrome. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A
treatment clinical trial is a research study meant to help improve current
treatments or obtain information on new treatments for patients with cancer. When clinical trials show that
a new treatment is better than the standard
treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Five types of standard treatment are used:
Photodynamic therapy
Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients undergoing photodynamic therapy will need to limit the amount of time spent in sunlight.
In one
type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug
called psoralen and then ultraviolet radiation is directed to
the skin. In another type of photodynamic therapy, called extracorporeal photochemotherapy, the patient is given drugs and then some blood cells are
taken from the body, put under a special ultraviolet A light, and put back into the body.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.
Sometimes, total skin electron beam (TSEB) radiation therapy is used to treat mycosis fungoides and the Sézary syndrome. This is a type of radiation treatment in which the skin over the whole body is treated with rays of tiny particles called electrons.
The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Sometimes the chemotherapy is topical (applied to the skin in a cream or lotion.) The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Other drug therapy
Retinoids, are drugs related to vitamin A that can slow the growth of certain types of cancer cells. The retinoids may be taken by mouth or applied to the skin.
Biologic therapy
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Specific types of biologic therapy used in treating mycosis
fungoides and the Sézary syndrome include the following:
- Monoclonal antibody therapy: A cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
- Interferon alfa:
A substance that interferes with the division of cancer cells and can slow
tumor growth.
- Interleukin-2: A
substance that can improve the body's natural response to infection and
disease.
New types of treatment are being tested in clinical
trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
High-dose chemotherapy and radiation therapy with stem cell transplant
This treatment is a method of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Back to Top Treatment Options by Stage
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Stage I Mycosis Fungoides and the Sézary Syndrome
Treatment of stage I mycosis fungoides and the Sézary syndrome may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Stage II Mycosis Fungoides and the Sézary Syndrome
Treatment of stage II mycosis fungoides and the Sézary syndrome is palliative and may
include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Stage III Mycosis Fungoides and the Sézary Syndrome
Treatment of stage III mycosis fungoides and the Sézary syndrome is palliative and may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Stage IV Mycosis Fungoides and the Sézary Syndrome
Treatment of stage IV mycosis fungoides and the Sézary syndrome is palliative and may
include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Back to Top Treatment Options for Recurrent Mycosis Fungoides and the Sézary
Syndrome
Treatment of recurrent mycosis fungoides and the Sézary syndrome may include the
following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Back to Top To Learn More About Mycosis Fungoides and the Sézary Syndrome
For more information from the National Cancer Institute about mycosis fungoides and the Sézary syndrome, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
Back to Top Get More Information From NCI
Call 1-800-4-CANCER
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.
Chat online
The NCI's LiveHelp® online chat service 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 to us
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
Search the NCI Web site
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. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find 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.
Back to Top Changes to This Summary (05/06/2008)
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.
Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:
- Text describing the way cancer spreads in the body was added to the Stages section.
- Information about patients taking part in clinical trials and about follow-up tests was added to the Treatment Option Overview section.
- Links to ongoing clinical trials listed in NCI's PDQ Cancer Clinical Trials Registry were added to the Treatment Options section.
- A new section called "To Learn More" was added. It includes links to more information about this type of cancer and about cancer in general.
- The "Get More Information from NCI" section (originally called "To Learn More") was revised.
Back to Top About PDQ
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.
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 the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
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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