Sézary's disease

Sézary's disease (often named Sézary syndrome) is a type of cutaneous lymphoma which 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. 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:

1. Generalized erythroderma
Lymphadenopathy
3. Atypical T-cells ("Sézary cells") in the peripheral blood
Hepatosplenomegaly

Diagnosis

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.

Treatment

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

Epidemiology

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. Patients with Sézary disease have a median survival of 5 years.