Sézary's disease

Template:DiseaseDisorder infobox Template:Sezary's disease Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Cutaneous T-cell lymphoma; mycosis fungoides; sezary syndrome

Overview

Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary. . "Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides. 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

1. Generalized erythroderma
2. Lymphadenopathy
3. 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.

External links

• Illustration of Sezary cells
• Biography of Sezary (in French)
• Sezary Syndrome lymphoma information
• Doctor's doctor
• Cutaneous Lymphoma Foundation
• [http://www.clinicaltrials.gov/ct/show/NCT00106431 Clinical Trial for Sezary Syndrome

• Template:GPnotebook

de:Sézary-Syndrom

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