• Mycosis fungoides and the Sézary syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin.
  • Mycosis fungoides and Sézary syndrome are types of cutaneous T-cell lymphoma.
  • A sign of mycosis fungoides is a red rash on the skin.
  • In Sézary syndrome, cancerous T-cells are found in the blood.
  • Tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and Sézary syndrome.
  • Certain factors affect prognosis (chance of recovery) and treatment options.
Mycosis fungoides and 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.

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

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

Mycosis fungoides and Sézary syndrome are the two most common types of cutaneous T-cell lymphoma (a type of non-Hodgkin lymphoma).

In 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 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 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:
    • The number of red blood cells and platelets.
    • The number and type of white blood cells.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • 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).
  • The patient's age and gender.

Mycosis fungoides and 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.

Stages of Mycosis Fungoides (Including Sézary Syndrome)

  • After mycosis fungoides and 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.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • The following stages are used for mycosis fungoides and Sézary syndrome:
    • Stage I Mycosis Fungoides
    • Stage II Mycosis Fungoides
    • Stage III Mycosis Fungoides
    • Stage IV Mycosis Fungoides
    • Stage IV Sézary Syndrome
After mycosis fungoides and 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 Sézary syndrome:
Stage I Mycosis Fungoides

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, papules, and/or plaques.
  • Stage IB: Ten percent or more of the skin surface is covered with patches, papules, and/or plaques.

There may be abnormal lymphocytes in the blood but they are not cancerous.

Stage II Mycosis Fungoides

Stage II is divided into stage IIA and stage IIB as follows:

  • Stage IIA: Any amount of the skin surface is covered with patches, papules, and/or plaques. Lymph nodes are enlarged but cancer has not spread to them.
  • Stage IIB: One or more tumors that are 1 centimeter or larger are found on the skin. Lymph nodes may be enlarged but cancer has not spread to them.

There may be abnormal lymphocytes in the blood but they are not cancerous.

Stage III Mycosis Fungoides

In stage III, nearly all of the skin is reddened and may have patches, papules, plaques, or tumors. Lymph nodes may be enlarged but cancer has not spread to them.

There may be abnormal lymphocytes in the blood but they are not cancerous.

Stage IV Mycosis Fungoides

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, papules, plaques, or tumors, and either:
    • cancer has spread to lymph nodes and there may be cancerous lymphocytes in the blood; or
    • there are cancerous lymphocytes in the blood and lymph nodes may be enlarged, but cancer has not spread to them.
  • Stage IVB: Most of the skin is reddened and any amount of the skin surface is covered with patches, papules, plaques, or tumors. Cancer has spread to other organs in the body. Lymph nodes may be enlarged and cancer may have spread to them. There may be cancerous lymphocytes in the blood.
Stage IV Sézary Syndrome

In stage IV:

  • Most of the skin is reddened and covered with patches, papules, plaques, or tumors; and
  • There is a high level of cancerous lymphocytes in the blood; and
  • Lymph nodes may be enlarged and cancer may have spread to them.

Recurrent Mycosis Fungoides (Including Sézary Syndrome)

Recurrent mycosis fungoides and 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.

Treatment Option Overview

  • There are different types of treatment for patients with mycosis fungoides and Sézary syndrome cancer.
  • Five types of standard treatment are used:
  • New types of treatment are being tested in clinical trials.
  • Treatment for mycosis fungoides and Sézary syndrome may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.
There are different types of treatment for patients with mycosis fungoides and Sézary syndrome cancer.

Different types of treatment are available for patients with mycosis fungoides and 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. Extracorporeal photochemotherapy may be used alone or combined with total skin electron beam (TSEB) radiation therapy.

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.

The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat mycosis fungoides and Sézary syndrome, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Sometimes, total skin electron beam (TSEB) radiation therapy is used to treat mycosis fungoides and Sézary syndrome. This is a type of external radiation treatment in which a radiation therapy machine aims electrons (tiny, invisible particles) at the skin covering the whole body.

Ultraviolet B (UVB) radiation therapy or ultraviolet A (UVA) radiation therapy may be given using a special lamp or laser that directs radiation at the skin.

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 cerebrospinal fluid, 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 (put on the skin in a cream, lotion, or ointment). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Other drug therapy

Topical corticosteroids are used to relieve red, swollen, and inflamed skin. They are a type of steroid. Topical corticosteroids may be in a cream, lotion, or ointment.

Retinoids, such as bexarotene, 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 put on the skin.

Lenalidomide is a drug that helps the immune system kill abnormal blood cells or cancer cells and may prevent the growth of new blood vessels that tumors need to grow.

Vorinostat and romidepsin are two of the histone deacetylase (HDAC) inhibitors used to treat mycosis fungoides and Sézary syndrome. HDAC inhibitors cause a chemical change that stops tumor cells from dividing.

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.

Interferon is a type of biologic therapy used to treat mycosis fungoides and Sézary syndrome. It interferes with the division of cancer cells and can slow tumor growth.

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.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

Brentuximab vedotin is a type of targeted therapy being studied in the treatment of mycosis fungoides and Sézary syndrome. It is made up of a monoclonal antibody linked to an anticancer drug.

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 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.

Treatment Options by Stage

Stage I and Stage II Mycosis Fungoides

Treatment of stage I and stage II mycosis fungoides may include the following:

  • Psoralen and ultraviolet A (PUVA) radiation therapy.
  • Radiation therapy with total skin electron beam radiation therapy or ultraviolet B radiation. In some cases, radiation therapy is given to skin lesions, as palliative therapy to reduce tumor size to relieve symptoms and improve quality of life.
  • Biologic therapy given alone or combined with therapy directed at the skin.
  • Topical chemotherapy.
  • Systemic chemotherapy with one or more drugs, which may be combined with therapy directed at the skin.
  • Other drug therapy (topical corticosteroids, retinoid therapy, lenalidomide, histone deacetylase inhibitors).
  • A clinical trial of targeted therapy with brentuximab vedotin.
Stage III and Stage IV Mycosis Fungoides (Including Sézary Syndrome)

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

  • Psoralen and ultraviolet A (PUVA) radiation therapy.
  • Extracorporeal photochemotherapy given alone or combined with total skin electron beam radiation therapy.
  • Radiation therapy with total skin electron beam radiation therapy or ultraviolet B radiation and ultraviolet A radiation. In some cases, radiation therapy is given to skin lesions, as palliative therapy to reduce tumor size to relieve symptoms and improve quality of life.
  • Biologic therapy given alone or combined with therapy directed at the skin.
  • Systemic chemotherapy with one or more drugs, which may be combined with therapy directed at the skin.
  • Topical chemotherapy.
  • Other drug therapy (topical corticosteroids, lenalidomide, bexarotene, histone deacetylase inhibitors).
  • A clinical trial of targeted therapy with brentuximab vedotin.

Treatment Options for Recurrent Mycosis Fungoides (Including Sézary Syndrome)

Treatment of recurrent mycosis fungoides including Sézary syndrome may be within a clinical trial and may include the following:

  • Radiation therapy with total skin electron beam radiation therapy or ultraviolet B (UVB). In some cases, radiation therapy is given to skin lesions as palliative therapy to reduce tumor size to relieve symptoms and improve quality of life.
  • Psoralen and ultraviolet A (PUVA) radiation therapy, which may be given with biologic therapy.
  • Extracorporeal photochemotherapy.
  • Systemic chemotherapy with one or more drugs.
  • Other drug therapy (topical corticosteroids, retinoid therapy, lenalidomide, histone deacetylase inhibitors).
  • Biologic therapy given alone or combined with therapy directed at the skin
  • A clinical trial of high-dose chemotherapy with stem cell transplant.
  • A clinical trial of targeted therapy with brentuximab vedotin.

Source: National Institutes of Health; National Cancer Institute

This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 09/07/2017...#8493