Sézary syndrome This 61-year-old man presented in 1972 with unrelenting pruritus of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).
|Classification and external resources|
|eMedicine||med/1541 derm/566 med/3486|
Sézary's disease (often called Sézary syndrome) (//) is a type of cutaneous lymphoma that was first described by Albert Sézary. The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy. There are currently no known causes of Sézary's disease.
Signs and symptoms
Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:
- Generalized erythroderma
- Atypical T-cells ("Sézary cells") in the peripheral blood
Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a biopsy of a skin lesion can be performed to rule out any other causes.
Vorinostat (Zolinza) is a second-line drug for cutaneous T-cell lymphoma. Treatments are often used in combination with phototherapy and chemotherapy. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age.
- synd/3594 at Who Named It?
- Cuneo A, Castoldi. "Mycosis fungoidses/Sezary's syndrome". Retrieved 2008-02-15.
- Thangavelu, Maya. "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Retrieved 2008-02-15.
- "Causes and Symptoms". Retrieved 2008-02-15.
- Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2.
- Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
- "Diagnosis". Retrieved 2008-02-15.
- Illustration of Sezary cells
- Biography of Sezary (in French)
- Sezary Syndrome lymphoma information
- Doctor's doctor
- Cutaneous Lymphoma Foundation
- Clinical Trial for Sezary Syndrome
- Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan