Sézary disease: Difference between revisions
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Revision as of 11:52, 26 June 2013
| Sézary disease | |
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| Specialty | Oncology  |
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]
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]
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]
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]
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][6]
See also
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 2008-02-15.
- ^ Thangavelu, Maya. "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Retrieved 2008-02-15.
- ^ "Causes and Symptoms". Retrieved 2008-02-15.
- ^ a b Cerroni, Lorenzo (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2.
{{cite book}}: Unknown parameter|coauthors=ignored (|author=suggested) (help) Cite error: The named reference "Skin Lymphomas 2004" was defined multiple times with different content (see the help page). - ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
- ^ a b c "Diagnosis". Retrieved 2008-02-15.
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
- Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan