Last Modified : 2005-11-02
Lymphocytes are made in the bone marrow and fight infection and disease. There are three types of lymphocytes:
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's lymphoma, refer to the following PDQ summaries:
Mycosis fungoides and the Sézary syndrome may move through the following phases:
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:
The prognosis (chance of recovery) and treatment options depend on the following:
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.
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:
Stage I is divided into stage IA and stage IB as follows:
Stage II is divided into stage IIA and stage IIB as follows:
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 IVStage IV is divided into stage IVA and stage IVB as follows:
The B classification is based on how many abnormal lymphocytes are found in the blood.
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.
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. Before starting treatment, patients may want to think about taking part in a clinical trial. 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.
Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Five types of standard treatment are used: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 therapyRadiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. 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.
ChemotherapyChemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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 therapyRetinoids 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 therapyBiologic 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:
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.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Treatment of stage I mycosis fungoides and the Sézary syndrome may include the following:
Treatment of stage II mycosis fungoides and the Sézary syndrome is palliative and may include the following:
Treatment of stage III mycosis fungoides and the Sézary syndrome is palliative and may include the following:
Treatment of stage IV mycosis fungoides and the Sézary syndrome is palliative and may include the following:
Treatment of recurrent mycosis fungoides and the Sézary syndrome may include the following:
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.