Sezary syndrome
Sezary syndrome (pronunciation: suh-ZAR-ee) is a rare form of cutaneous T-cell lymphoma characterized by the widespread redness and scaling of the skin (erythroderma) and the presence of malignant T cells (called Sezary cells) in the blood.
Etymology
The syndrome is named after the French dermatologist Albert Sézary, who first described the condition in 1938.
Symptoms
The most common symptoms of Sezary syndrome include:
- Erythroderma: A red, itchy rash that covers most of the body
- Lymphadenopathy: Enlarged lymph nodes
- Alopecia: Hair loss
- Onychodystrophy: Abnormalities of the nails
- Hepatosplenomegaly: Enlargement of the liver and spleen
Diagnosis
Diagnosis of Sezary syndrome is based on the presence of specific symptoms, a physical examination, and specialized tests. These tests may include a skin biopsy, blood tests to identify Sezary cells, and imaging tests to check for enlarged lymph nodes or organs.
Treatment
Treatment options for Sezary syndrome may include skin-directed therapy, systemic therapy, or a combination of both. Skin-directed therapies include topical corticosteroids, retinoids, and phototherapy. Systemic therapies include chemotherapy, biological therapy, and targeted therapy.
Prognosis
The prognosis for individuals with Sezary syndrome varies. Factors that can affect prognosis include the stage of the disease, the individual's overall health, and their response to treatment.
See also
External links
- Medical encyclopedia article on Sezary syndrome
- Wikipedia's article - Sezary syndrome
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