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 become mature blood stem cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes a red blood cell, white blood cell, or platelet. A lymphoid stem cell becomes a lymphoblast and then one of three types of lymphocytes (white blood cells):

• B-cell lymphocytes that make antibodies to help fight infection.
• T-cell lymphocytes that help B-lymphocytes make the antibodies that help fight infection.
• Natural killer cells that attack cancer cells and viruses.

Figure

Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell.

In mycosis fungoides, T-cell lymphocytes become cancerous and affect the skin. In the Sézary syndrome, cancerous T-cell lymphocytes affect the skin and are in the blood.

Mycosis fungoides and the Sézary syndrome are types of cutaneous T-cell lymphoma.

Mycosis fungoides and the Sézary syndrome are the two most common types of cutaneous T-cell lymphoma (a type of non-Hodgkin lymphoma). For information about other types of skin cancer or non-Hodgkin lymphoma, see the following PDQ summaries:

• Adult Non-Hodgkin Lymphoma Treatment
• Skin Cancer Treatment
• Melanoma Treatment
• Kaposi Sarcoma Treatment

A sign of mycosis fungoides is a red rash on the skin.

Mycosis fungoides may go 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: Small raised bumps (papules) or hardened lesions on the skin, which may be reddened.
• Tumor phase: Tumors form on the skin. These tumors may develop ulcers and the skin may get infected.

Check with your doctor if you have any of these signs.

In the Sézary syndrome, cancerous T-cells are found in the blood.

Also, skin all over the body is reddened, itchy, peeling, and painful. There may also be patches, plaques, or tumors on the skin. It is not known if the Sézary syndrome is an advanced form of mycosis fungoides or a separate disease.

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:

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  The number of red blood cells and platelets.

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  The number and type of white blood cells.

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  The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

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  The portion of the blood sample made up of red blood cells.

  

  Figure

  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 (more...)

• 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.
• Skin 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.
• T-cell receptor (TCR) gene rearrangement test: A laboratory test in which cells in a sample of tissue are checked to see if there is a certain change in the genes. This gene change can lead to too many of one kind of T-cells (white blood cells that fight infection) to be made.
• Flow cytometry: A laboratory test that measures the number of cells in a sample of blood, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.

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 type of lesion (patches, plaques, or tumors).

Mycosis fungoides and the Sézary syndrome are hard to cure. Treatment is usually palliative, to relieve symptoms and improve the quality of life. Patients with early stage disease may live many years.

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.
• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
• 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.
• Bone marrow aspiration and biopsy: The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if mycosis fungoides spreads to the liver, the cancer cells in the liver are actually mycosis fungoides cells. The disease is metastatic mycosis fungoides, not liver cancer.

The following stages are used for mycosis fungoides and the Sézary syndrome:

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.

Six types of standard treatment are used:

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.

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 listing of clinical trials.

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.

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.

Stage I Mycosis Fungoides

Treatment of stage I mycosis fungoides may include the following:

• PUVA therapy with or without biologic therapy (interferon).
• Radiation therapy to a single skin lesion or to all the skin on the body (TSEB).
• Topical corticosteroid therapy.
• Radiation therapy to skin lesions, as palliative therapy to reduce tumor size or relieve symptoms and improve quality of life.
• Topical chemotherapy.
• Biologic therapy (interferon) with or without topical chemotherapy.
• Retinoid therapy.
• Systemic chemotherapy.
• Targeted therapy with vorinostat, romidepsin, or other histone deacetylase (HDAC) inhibitors.
• Targeted therapy with pralatrexate.
• Targeted therapy with a monoclonal antibody (denileukin diftitox).
• A clinical trial of ultraviolet B (UVB) radiation therapy.
• A clinical trial of a new treatment.

Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.

Stage II Mycosis Fungoides

Treatment of stage II mycosis fungoides is palliative (to relieve symptoms and improve the quality of life) and may include the following:

• PUVA therapy with or without biologic therapy (interferon).
• Radiation therapy to all the skin on the body (TSEB).
• Topical corticosteroid therapy.
• Topical chemotherapy.
• Radiation therapy to skin lesions.
• Biologic therapy (interferon) with or without topical chemotherapy.
• Retinoid therapy.
• Oral chemotherapy.
• Systemic chemotherapy.
• Targeted therapy with vorinostat, romidepsin, or other histone deacetylase (HDAC) inhibitors.
• Targeted therapy with pralatrexate.
• Targeted therapy with a monoclonal antibody (denileukin diftitox).
• A clinical trial of ultraviolet B (UVB) radiation therapy.
• A clinical trial of a new treatment.

Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.

Stage III Mycosis Fungoides

Treatment of stage III mycosis fungoides is palliative (to relieve symptoms and improve the quality of life) and may include the following:

• PUVA therapy with or without systemic chemotherapy.
• PUVA therapy with or without biologic therapy (interferon).
• Radiation therapy to all the skin on the body (TSEB).
• Topical corticosteroid therapy.
• Radiation therapy to skin lesions.
• Systemic chemotherapy with one or more drugs, with or without topical chemotherapy.
• Biologic therapy (interferon) with or without topical chemotherapy.
• Extracorporeal photochemotherapy.
• Topical chemotherapy.
• Retinoid therapy.
• Oral chemotherapy.
• Targeted therapy with vorinostat, romidepsin, or other histone deacetylase (HDAC) inhibitors.
• Targeted therapy with pralatrexate.
• Targeted therapy with a monoclonal antibody (alemtuzumab or denileukin diftitox).
• A clinical trial of ultraviolet B (UVB) radiation therapy.
• A clinical trial of a new treatment.

Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.

Stage IV Mycosis Fungoides and the Sézary Syndrome

Treatment of stage IV mycosis fungoides and stage IV Sézary syndrome is palliative (to relieve symptoms and improve the quality of life) and may include the following:

• PUVA therapy with or without systemic chemotherapy.
• PUVA therapy with or without biologic therapy (interferon).
• Radiation therapy to all the skin on the body (TSEB), with or without systemic chemotherapy.
• Topical corticosteroid therapy.
• Radiation therapy to skin lesions.
• Systemic chemotherapy with one or more drugs.
• Biologic therapy (interferon) with or without topical chemotherapy.
• Targeted therapy with a monoclonal antibody (alemtuzumab or denileukin diftitox).
• Topical chemotherapy.
• Extracorporeal photochemotherapy with or without radiation therapy to all the skin on the body.
• Retinoid therapy.
• Oral chemotherapy.
• Targeted therapy with vorinostat, romidepsin, or other histone deacetylase (HDAC) inhibitors.
• Targeted therapy with pralatrexate.
• A clinical trial of ultraviolet B (UVB) radiation therapy.
• A clinical trial of a new treatment.

Check the list of NCI-supported cancer clinical trials 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. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

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Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of mycosis fungoides and the Sézary Syndrome. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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Clinical Trial Information

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.

Clinical trials are listed in PDQ and can be found online at NCI's website. 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).

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