Sézary's disease
Template:DiseaseDisorder infobox
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary. [1]
"Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides.
Sézary's disease is sometimes considered a late stage of mycosis fungoides.
General
Sezary syndrome and Mycosis Fungoides are T-cell lymphomas whose primary manifestation is in the skin.
Etiology
HTLV has thought to have been associated with both of the above cutaneous mycoses.
Symptoms
The hallmark symptoms for Sézary's disease are
- Generalized erythroderma
- Lymphadenopathy
- Atypical T-cells ("Sezary cells") in the peripheral blood.
Treatment
Vorinostat (Zolinza®) is a second-line drug for CTCL. Treatments are often used in combination.