Cutaneous T-cell lymphoma (CTCL) is a type of skin cancer. A type of white blood cell called T-cell lymphocytes that normally fight infections become cancerous and affect the skin.
What are mycosis fungoides and the Sezary syndrome?
Mycosis fungoides and the Sézary syndrome are the two most common forms of cutaneous T-cell lymphoma.
In mycosis fungoides, T-cells become cancerous and spread to the skin.
In the Sézary syndrome, T-cells become cancerous affect the skin and the blood.
What are the symptoms of CTCL?
Mycosis fungoides and the Sézary syndrome may evolve 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.
The Sézary syndrome is an advanced form of mycosis fungoides during which the skin all over the body is reddened, itchy, peeling, and painfu and cancerous T-cells are found in the blood.
Mycosis fungoides does not always progress to the Sézary syndrome.
How are mycosis fungoides and the Sézary syndrome diagnosed?
The following tests and procedures may be used to diagnose mycosis fungoides and Sézary syndrome:
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 CBC blood test checks for the number and type of lympocytes and other 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.
What factors affect prognosis 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.
How do doctors determine if the disease has spread?
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.
What are the stages of mycosis fungoides and the Sézary syndrome?
The following stages are used for mycosis fungoides and the Sézary syndrome:
- 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 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.
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 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, which is based on how many abnormal lymphocytes are found in the blood.
How is it treated?
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 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 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 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. These include 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.