Sézary syndrome is a rare type of lymphoma. It affects the skin, blood and sometimes the lymph nodes. The disease is most common in middle-aged and older people. Sézary syndrome progresses quickly once it develops. Treatments include photopheresis and immunotherapy, among others. Having Sézary syndrome increases your risk of other cancers.
Sézary syndrome is a rare cancer that affects the skin and the blood. It can lead to:
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.
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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.
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.
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).
Sézary syndrome can cause a variety of skin problems:
Other symptoms may include:
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.
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:
If your provider suspects Sézary syndrome, they may do the following tests:
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:
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:
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.
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:
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.
If you have Sézary syndrome, you may want to ask your healthcare provider:
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.
Last reviewed by a Cleveland Clinic medical professional on 04/10/2021.
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