Join the RareGuru Community
To connect, share, empower and heal today.
Don’t fight Sezary syndrome alone.
Find your community on the free RareGuru App.Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin. It is characterized by a widespread red rash that may cover most of the body, the presence of cancerous T cells (called Sezary cells) in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. The exact cause of Sezary syndrome is currently unknown. Treatment varies based on the signs and symptoms present in each person and the severity of the condition.
Source: GARD Last updated on 05-01-20
Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin. In Sezary syndrome, specifically, the cancerous T cells are called Sezary cells and are found in the skin, lymph nodes, and blood. They can also spread to other organs in the body, including the liver, spleen, and bone marrow.
Although Sezary syndrome can affect people of all ages, it is most commonly diagnosed in adults over age 60. The signs and symptoms of this condition can vary but may include:
Affected people may also have an increased risk of developing another lymphoma or other type of cancer.
Last updated on 05-01-20
Although a small percentage of cases of Sezary syndrome are associated with human T-lymphotropic viruses type 1 and type 2, the underlying cause of most cases is currently unknown. In people affected by the condition, the cancerous T-cells (also called Sezary cells) usually have one or more chromosomal abnormalities. These genetic changes are considered "somatic" because they are acquired during a person's lifetime, are not inherited and only affect the DNA of the cancerous cells. Although any chromosome can be affected, people with Sezary syndrome often have deletions of DNA from chromosomes 10 and 17 or duplications of DNA on chromosomes 8 and 17. However, it is unclear whether these alterations play a role in the development of the condition.
Last updated on 05-01-20
A diagnosis of Sezary syndrome is often suspected in people with characteristic signs and symptoms. Additional testing can then be ordered to confirm the diagnosis. This may include:
To learn more about the diagnosis of Sezary syndrome, including specific information about each of the tests listed above, please visit the National Cancer Institute's Web site. Simply click on the link and scroll down to the section entitled "tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Sezary syndrome"
Last updated on 05-01-20
The underlying cause of Sezary syndrome is unknown in most cases. However, it generally occurs sporadically in people with no family history of the condition.
Last updated on 05-01-20
The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, there are several clinical trials are identified as enrolling individuals with Sezary syndrome. To find these trials, click on the link above. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.
You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling 1-800-411-1222 to speak with a specialist, who can help you determine if you are eligible for any clinical trials. If you are located outside the United States, and would like to be contacted via telephone, you will need to contact PRPL and provide your telephone number in full, including area code and international dialing prefix.
Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 1-800-411-1222
Fax: 301-480-9793
E-mail: prpl@mail.cc.nih.gov
Web site: http://clinicalcenter.nih.gov
You can find information about participating in a clinical trial as well as learn about resources for travel and lodging assistance, through the Get Involved in Research section of our Web site.
Last updated on 05-01-20
The long-term outlook (prognosis) for people with Sezary syndrome is generally poor. Sezary syndrome is difficult to cure. Treatment is usually palliative, with the intention of relief of symptoms and improvement in the quality of life. Median survival for patients with Sezary syndrome has been reported to be 2 to 4 years after development of the condition, although survival has improved with newer treatments. The disease-specific 5-year survival rate has been reported to be 24%.
Last updated on 05-01-20
In general, there are six different treatment options available to people with Sezary syndrome. These include:
To learn more about the treatment and management of Sezary syndrome, including specific information about each of the treatments listed above, please visit the National Cancer Institute's Web site. Simply click on the link to access this resource.
Last updated on 05-01-20
Sezary syndrome is difficult to cure. Treatment is usually palliative, with the intention of relief of symptoms and improvement in the quality of life. Patients may live many years with the disease.
Last updated on 05-01-20
Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!
To connect, share, empower and heal today.