Sézary's disease
| Sézary disease | |
|---|---|
| Classification and external resources | |
 Sézary syndrome This 61-year-old man presented in 1972 with unrelenting pruritus of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell). |
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| 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 called Sézary syndrome) is a type of cutaneous lymphoma that 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 with lymphadenopathy.[2][3] There are currently no known causes of Sézary's disease.[4]
Contents |
[edit] Signs and symptoms
Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin.[5] 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[6]
[edit] Diagnosis
Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication.[7] 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.[7] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[7]
[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] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[5]
[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.[6]
[edit] See also
[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". http://atlasgeneticsoncology.org/Anomalies/MycosFungID2039.html. Retrieved 2008-02-15.
- ^ Thangavelu, Maya. "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". http://bloodjournal.hematologylibrary.org/cgi/content/full/89/9/3371. Retrieved 2008-02-15.
- ^ "Causes and Symptoms". http://www.sezary.com/sezary-syndrome-causes.html. Retrieved 2008-02-15.
- ^ a b Cerroni, Lorenzo; Kevin Gatter, Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. pp. 39. ISBN 978-1405113762.
- ^ a b Lorincz, A. I.. "Sezary syndrome". http://www.gpnotebook.co.uk/simplepage.cfm?ID=584056846. Retrieved 2008-02-15.
- ^ a b c "Diagnosis". http://www.sezary.com/sezary-syndrome-diagnosis.html. Retrieved 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
- Clinical Trial for Sezary Syndrome
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