Sézary disease
(Redirected from Sézary syndrome)
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| Sézary disease | |
|---|---|
| Synonyms | Sézary syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Erythroderma, lymphadenopathy, pruritus, alopecia, onychodystrophy, keratoderma |
| Complications | Infection, sepsis, organ failure |
| Onset | Typically in adults over 50 |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown, possibly genetic and environmental factors |
| Risks | Age, male gender, family history |
| Diagnosis | Blood test, skin biopsy, flow cytometry |
| Differential diagnosis | Psoriasis, eczema, drug reactions |
| Prevention | N/A |
| Treatment | Phototherapy, chemotherapy, biological therapy, stem cell transplant |
| Medication | N/A |
| Prognosis | Variable, often poor |
| Frequency | Rare |
| Deaths | N/A |
Sézary disease is a type of cutaneous T-cell lymphoma, a group of lymphomas that affect the skin. It is named after the French dermatologist Albert Sézary who first described the condition in 1938.
Symptoms[edit | edit source]
The most common symptoms of Sézary disease include redness of the skin (erythroderma), intense itching (pruritus), and enlarged lymph nodes (lymphadenopathy). Other symptoms may include changes in the nails, hair loss, and palmar and plantar keratoderma.
Causes[edit | edit source]
The exact cause of Sézary disease is unknown. However, it is believed to be caused by changes in the T cells, a type of white blood cell that plays a key role in the body's immune response.
Diagnosis[edit | edit source]
Diagnosis of Sézary disease is based on a combination of clinical findings, blood tests, and skin biopsy. The presence of Sézary cells, abnormal T cells with a characteristic appearance, in the blood is a key diagnostic feature.
Treatment[edit | edit source]
Treatment options for Sézary disease include skin-directed therapies, systemic therapies, and stem cell transplantation. The choice of treatment depends on the stage of the disease and the patient's overall health.
Prognosis[edit | edit source]
The prognosis for patients with Sézary disease varies widely. Factors that can affect prognosis include the stage of the disease, the patient's age and overall health, and the response to treatment.
See also[edit | edit source]
References[edit | edit source]
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Contributors: Prab R. Tumpati, MD
