Sezary syndrome

Sezary syndrome is a type of cutaneous T-cell lymphoma (CTCL), which belongs to a larger group of disorders known as non-HodgkinÕs 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. The prognosis is generally poor with a median survival between 2 and 4 years. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.