Sézary's disease
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| Sézary disease Classification and external resources |
|
| ICD-10 | C84.1 |
|---|---|
| ICD-9 | 202.2 |
| ICD-O: | M9701/3 |
| DiseasesDB | 8595 |
| eMedicine | med/1541 derm/566 med/3486 |
| MeSH | D012751 |
Sézary's disease (often named Sézary syndrome) is a type of cutaneous lymphoma which was first described by Albert Sézary.[1] The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides.[2][3] There are currently no known causes of Sézary's disease.[4]
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[edit] 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[2], although rarer CD8+/CD4- cases have been observed.[2] Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease.[2] The dominant symptoms of the disease are:
- Generalized erythroderma[2]
- Lymphadenopathy[2]
- Atypical T-cells ("Sézary cells") in the peripheral blood[2]
- Hepatosplenomegaly[5]
[edit] Diagnosis
Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication.[6] 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.[6] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[6]
[edit] Treatment
Vorinostat (Zolinza) is a second-line drug for cutaneous T-cell lymphoma.[citation needed] Treatments are often used in combination with phototherapy and chemotherapy.[2]
[edit] Epidemiology
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma.[2] In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people.[2] Sézary disease is more common in males with a ratio of 2:1,[2] and the mean age of diagnosis is between 55 and 60 years of age.[2] Patients with Sézary disease have a median survival of 5 years.[5]
[edit] References
- ^ synd/3594 at Who Named It
- ^ a b c d e f g h i j k l Cuneo A, Castoldi. Mycosis fungoidses/Sezary's syndrome. Retrieved on 2008-02-15.
- ^ Thangavelu, Maya. Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome. Retrieved on 2008-02-15.
- ^ Causes and Symptoms. Retrieved on 2008-02-15.
- ^ a b Lorincz, A. I.. Sezary syndrome. Retrieved on 2008-02-15.
- ^ a b c Diagnosis. Retrieved on 2008-02-15.
[edit] External links
- Illustration of Sezary cells
- 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
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