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):
- 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 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.
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:
-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.
- 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.
Stages of Mycosis Fungoides and the Sézary Syndrome
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 IIA: Any amount of the skin surface is covered with
patches and/or plaques. Lymph nodes are enlarged but cancer has not spread to them.
- Stage IIB: One or more tumors are found on the skin. Lymph
nodes may be enlarged but cancer has not spread to them.
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.
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.
Treatment Option Overview
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.
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.
Treatment Options by Stage
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:
- PUVA therapy with or without interferon alfa.
- Radiation therapy to a single skin lesion or to all the skin
on the body (TSEB).
- Radiation therapy to skin lesions, as palliative therapy to
reduce tumor size or relieve symptoms and improve quality of life.
- Topical chemotherapy
- Interferon alfa with or without topical chemotherapy.
- Retinoid therapy
- Systemic chemotherapy
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:
- PUVA therapy with or without interferon alfa.
- Radiation therapy to all the skin on the body (TSEB).
- Radiation therapy to skin lesions, to reduce tumor size or
relieve symptoms and improve quality of life.
- Topical chemotherapy
- Interferon alfa with or without topical chemotherapy.
- Retinoid therapy
- Systemic chemotherapy
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:
- PUVA therapy with or without systemic chemotherapy.
- PUVA therapy with or without interferon alfa.
- Extracorporeal photochemotherapy.
- Radiation therapy to all the skin on the body (TSEB).
- Radiation therapy to skin lesions, to reduce tumor size or
relieve symptoms and improve quality of life.
- Systemic chemotherapy with one or more drugs, with or
without topical chemotherapy.
- Topical chemotherapy
- Biologic therapy (interferon alfa or interleukin-2) with or
without topical chemotherapy.
- Retinoid therapy
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:
- PUVA therapy with or without systemic chemotherapy.
- PUVA therapy with or without interferon alfa.
- Extracorporeal photochemotherapy with or without radiation
therapy to all the skin on the body (TSEB).
- Radiation therapy to all the skin on the body, with or
without systemic chemotherapy.
- Radiation therapy to skin lesions.
- Systemic chemotherapy with one or more drugs.
- Biologic therapy (interferon alfa or interleukin-2) with or
without topical chemotherapy.
- Topical chemotherapy
- Monoclonal antibody therapy.
- Retinoid therapy
Treatment Options for Recurrent Mycosis Fungoides and the Sézary Syndrome
Treatment of recurrent mycosis fungoides and the Sézary syndrome
may include the following:
- Radiation therapy to skin lesions or all the skin on the body (TSEB).
- PUVA therapy.
- Topical chemotherapy.
- A clinical trial of PUVA therapy with interferon alfa.
- A clinical trial of extracorporeal photochemotherapy.
- A clinical trial of radiolabeled monoclonal antibody therapy.
- A clinical trial of interleukin-2.
- A clinical trial of retinoid therapy.
- A clinical trial of chemotherapy.
- A clinical trial of high-dose chemotherapy with stem cell transplant.
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
U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 800.4.CANCER (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 800.332.8615.
Information about ongoing clinical trials is available from the NCI Web site.
Source: National Institutes of Health; National Cancer Institute
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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: 5/6/2008...#8493
