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Sezary syndrome
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Overview
Sezary syndrome is a type of cutaneous T-cell lymphoma (CTCL), which belongs to a larger group of disorders known as non-Hodgkin’s lymphomas. Sezary syndrome is characterized by a widespread red rash that may cover most of the body, the presence of specific malignant cells (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 cutaneous T-cell lymphomas is currently unknown. Treatment options vary depending on severity and signs and symptoms but may include topical chemotherapy, radiation therapy, photochemotherapy, use of retinoids, and chemotherapy.[1] The prognosis is generally poor with a median survival between 2 and 4 years.[2]
References
- Cutaneous T-Cell Lymphomas. NORD. http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1124/viewAbstract. Accessed April 30, 2012.
- Vanessa Ngan. Cutaneous T-cell lymphoma. DermNet NZ. http://www.dermnetnz.org/dermal-infiltrative/cutaneous-t-cell-lymphoma.html. Accessed April 30, 2012.
Your Questions Answered
by the Genetic and Rare Diseases Information Center2 question(s) from the public on Sezary syndrome have been answered. See questions and answers. You can also submit a new question.
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General Information
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. Click on the link to view the information.
- Genetics Home Reference (GHR) contains information on Sezary syndrome. Click on the link to go to GHR and review the information.
- Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
- The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Sezary syndrome. Click on the link to view a sample search on this topic.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Sezary syndrome. Click on the link to go to OMIM and review these resources.