Sézary’s disease

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Sézary’s disease (often named Sézary syndrome) is a type of cutaneous lymphoma which was first described by Albert Sézary.[1] The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary’s disease is sometimes considered a late stage of mycosis fungoides.[2][3] There are currently no known causes of Sézary’s disease.[4]

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[2], although rarer CD8+/CD4- cases have been observed.[2] Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier’s microabscesses is the hallmark sign of the disease.[2] The dominant symptoms of the disease are:

Patients who have Sézary’s disease often present with skin lesions that do not heal with normal medication.[6] 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.[6] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[6]

Vorinostat (Zolinza) is a second-line drug for cutaneous T-cell lymphoma.[citation needed] Treatments are often used in combination with phototherapy and chemotherapy.[2]

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma.[2] In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people.[2] Sézary disease is more common in males with a ratio of 2:1,[2] and the mean age of diagnosis is between 55 and 60 years of age.[2] Patients with Sézary disease have a median survival of 5 years.[5]

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