Mycosis Fungoides and the Sézary Syndrome
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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.
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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:
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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.
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Stage II
Either of the following may be true:
- The skin has red, dry, scaly patches, but no tumors. Lymph nodes are larger
than normal, but do not contain cancer cells.
- There are tumors on the skin. The lymph nodes are either normal or are
larger than normal, but do not contain cancer cells.
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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.
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Stage IV
The skin is involved, in addition to either of the following:
- Cancer cells are found in the lymph nodes.
- Cancer has spread to other organs, such as the liver or lung.
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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.
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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.
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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.
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Stage I Mycosis Fungoides/Sézary Syndrome
Treatment may be one of the following:
- Phototherapy (PUVA therapy) with or without biological therapy.
- Total skin electron beam radiation therapy (TSEB radiation therapy).
- Topical chemotherapy.
- Local electron beam or x-ray therapy to reduce the size of the tumor or
to relieve symptoms.
- Interferon alfa (biological therapy) alone or in combination with topical
therapy.
- Retinoids.
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Stage II Mycosis Fungoides/Sézary Syndrome
Treatment may be one of the following:
- Phototherapy (PUVA therapy) with or without biological therapy.
- Total skin electron beam radiation therapy (TSEB radiation therapy).
- Topical chemotherapy.
- Local electron beam or x-ray therapy.
- Interferon alfa (biological therapy) alone or in combination with topical
therapy.
- Retinoids.
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Stage III Mycosis Fungoides/Sézary Syndrome
Treatment may be one of the following:
- Phototherapy (PUVA therapy) with or without biological therapy.
- Total skin electron beam radiation therapy (TSEB radiation therapy).
- Local electron beam or x-ray therapy.
- Chemotherapy for mycosis fungoides and Sézary syndrome.
- Interferon alfa (biological therapy) alone or in combination with topical
therapy.
- Biological therapy.
- Systemic chemotherapy with or without therapy to the skin.
- Extracorporeal photochemotherapy.
- Topical chemotherapy.
- Retinoids.
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Stage IV Mycosis Fungoides/Sézary Syndrome
Treatment may be one of the following:
- Phototherapy (PUVA therapy) with or without biological therapy.
- Total skin electron beam radiation therapy (TSEB radiation therapy).
- Local electron beam or x-ray therapy.
- Chemotherapy for mycosis fungoides and Sézary syndrome.
- Interferon alfa (biological therapy) alone or in combination with topical
therapy.
- Biological therapy.
- Systemic chemotherapy with or without therapy to the skin.
- Topical chemotherapy.
- Extracorporeal photochemotherapy.
- Monoclonal antibody therapy.
- Retinoids.
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Recurrent Mycosis Fungoides/Sézary Syndrome
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:
- Local electron beam or x-ray therapy.
- Total skin electron beam radiation therapy (TSEB radiation therapy).
- Phototherapy (PUVA therapy).
- Topical chemotherapy.
- Systemic chemotherapy.
- Extracorporeal photochemotherapy.
- Clinical trials of biological therapy.
- Clinical trials of bone marrow transplantation.
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Changes to This Summary (06/06/2003)
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.
This information is provided from the PDQ® database in collaboration with the NCI.
