Sézary Syndrome

What is Sézary syndrome?

Sézary syndrome is a rare cancer that affects the skin and the blood. It can lead to:

• Cancerous cells in the blood.
• Enlarged lymph nodes.
• Extensive skin rashes or lesions (tumor or other skin abnormalities).

Sézary syndrome is a type of chronic (ongoing) cutaneous T-cell lymphoma (CTCL). CTCLs cause lymphocytes (white blood cells) called T-cells to multiply out of control. CTCLs are a type of non-Hodgkin’s lymphoma.

Other CTCLs, such as mycosis fungoides, mostly affect the skin in the form of a red rash. But in Sézary syndrome, cancerous lymphocytes (Sézary cells) spread from the skin to the blood. The cancer can also travel to the lymph nodes and other areas of the body.

Sézary syndrome is an aggressive disease, meaning it spreads fast. Treatment can help manage the effects of the disease, but there’s no cure.

Who is at risk of getting Sézary syndrome?

Sézary syndrome usually affects people over the age of 50. It’s slightly more common in men than women. Most people with Sézary syndrome have no family history of the disease.

How common is Sézary syndrome?

CTCLs are rare, there are only about 3,000 cases per year. Sézary syndrome is the second most common CTCL, after mycosis fungoides. Sézary syndrome accounts for about 15% of all new CTCL cases.

What causes Sézary syndrome?

Experts don’t know the exact cause of Sézary syndrome. Some people with the disease have a human T-cell leukemia virus, which affects lymphocytes. These viruses involve changes to DNA, but they aren’t inherited (passed from parents to children).

What are the symptoms of Sézary syndrome?

Sézary syndrome can cause a variety of skin problems:

• Dry, peeling or itchy skin.
• Red rash over much of the body (usually at least 80% of the body involves a rash, which is termed “erythroderma”).
• Skin tumors.
• Thickened skin on the palms of the hands and soles of the feet.

Other symptoms may include:

• Abnormal nail growth.
• Alopecia (hair loss).
• Ectropion (lower eyelids turn outward, away from the eye).
• Edema (swelling in the arms or legs due to fluid buildup).
• Enlarged liver.
• Enlarged spleen.
• Fever.
• Problems controlling body temperature.
• Swollen lymph nodes.
• Unexplained weight loss.

What are the complications of Sézary syndrome?

The cancer can spread to the lungs, liver, spleen and bone marrow. People with Sézary syndrome are at a higher risk of developing other types of lymphoma or cancers. The disease can also lower the function of your immune system, increasing the risk of infections.

How is Sézary syndrome diagnosed?

Healthcare providers specializing in lymphomas can diagnose Sézary syndrome. Because it’s such a rare disease, a hematopathologist or a dermatopathologist should confirm the diagnosis. Your healthcare provider:

• Evaluates your symptoms.
• Performs a physical exam.
• Reviews your medical history.

If your provider suspects Sézary syndrome, they may do the following tests:

• Biopsy: A healthcare provider does a biopsy of your skin, lymph nodes or bone marrow. A biopsy checks a sample of tissue for signs of cancer.
• Blood tests: A complete blood count and peripheral blood smear check your red and white blood cells, platelets and hemoglobin. Blood flow cytometry can detect cancerous cells in your blood .
• Immunophenotyping: A blood or tissue sample can reveal markers present on cell surfaces. Markers indicate specific types of lymphomas.
• T-cell receptor (TCR) gene rearrangement test: A blood or bone marrow test looks for problems in the genes that control T-cell function.

Are Sézary syndrome and mycosis fungoides the same disease?

Some experts consider Sézary disease the most advanced stage of mycosis fungoides. Others consider it a separate disease. Sézary syndrome and stage IV mycosis fungoides share similar characteristics:

• Skin rash, tumors or lesions on 80% of the body.
• High numbers of Sézary cells in the blood.
• Cancer that has spread to the lymph nodes, liver, spleen or other organs.

How is Sézary syndrome treated?

Treatment for Sézary syndrome is usually palliative. This means treatment eases symptoms, but doesn’t cure the cancer.

Your healthcare provider recommends treatments based on the stage of the cancer and its symptoms. Treatment may include:

• Extracorporeal photopheresis (ECP): Photopheresis removes blood from your body and treats the cancerous cells with a drug that makes them sensitive to light. UV light then kills the harmful cells before blood goes back to your body.
• Immunotherapy: Immunotherapy stimulates your body’s immune system to fight cancer cells. It’s also called biological therapy.
• Other drug therapies: Corticosteroids can be in the form of creams or ointments that relieve red, irritated skin. Retinoid creams or gels can slow cancer cell growth. Histone deacetylase inhibitors stop tumor cells from dividing.
• UV Phototherapy: Ultraviolet light in the B range (UVB) or A range (UVA) is often used. A specific therapy called PUVA, which involves taking a drug called psoralen and then being exposed to UVA phototherapy, is sometimes used.
• Radiation therapy: You may receive total skin electron beam radiation therapy. It delivers powerful doses of radiation to the entire surface of your skin.
• Targeted therapy: Targeted therapy uses drugs to target proteins or genes that control how cancer cells grow and survive. For example, some drugs attack proteins found in lymphoma cells.
• Chemotherapy: You may receive chemotherapy topically (on the skin) or systemically (throughout your body) usually as a pill or through an IV. Traditional chemotherapies used for other lymphomas and leukemias are typically avoided if possible, though.

Your healthcare provider can give you more information about treatments available through clinical trials. Clinical trials test the effectiveness of the latest drugs and therapies.

How can I prevent Sézary syndrome?

Sézary syndrome doesn’t have a clear cause, so there’s currently no way to prevent the disease. But you can reduce your risk by avoiding the human T-lymphotropic virus. This virus spreads through:

• Blood transfusions.
• Breastfeeding.
• Sexual contact.
• Sharing needles.

What is the outlook for people with Sézary syndrome?

About 24% of people with Sézary syndrome survive for at least five years after the disease develops. This rate will improve as new, more effective treatments emerge.

What should I ask my healthcare provider?

If you have Sézary syndrome, you may want to ask your healthcare provider:

• What is the cancer’s stage?
• What treatments are right for me?
• What side effects can I expect from treatment?
• Are there clinical trials I can take part in?
• Can the cancer come back after treatment?

A note from Cleveland Clinic

Sézary syndrome is a rare type of lymphoma that affects the skin, blood and sometimes the lymph nodes. It’s an aggressive cancer, so it spreads quickly. Sézary syndrome causes widespread skin problems, a lowered immune system and other symptoms. It also increases the risk of other lymphomas and cancers.