Sézary disease
| Sézary disease | |
|---|---|
| File:Sézary's disease.jpg
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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| Classification and external resources | |
| Specialty | Oncology |
| ICD-10 | C84.1 |
| ICD-9-CM | 202.2 |
| ICD-O | M9701/3 |
| DiseasesDB | 8595 |
| eMedicine | med/1541 derm/566 med/3486 |
| Patient UK | Sézary disease |
| MeSH | D012751 |
Sézary disease (also known as Sézary's disease or Sézary('s) syndrome) (/ˌseɪˌzɑːˈriː/) 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 disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[2][3] There are currently no known causes of Sézary disease.[4]
Contents
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
Those who have Sézary 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 is a second-line drug for cutaneous T-cell lymphoma.[8] 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 Mycosis fungiodes per 100,000 people. 5% of these cases present as a primary Sézary syndrome[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
- ↑ Sézary's cell at Who Named It?
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 Lua error in package.lua at line 80: module 'strict' not found.
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External links
- Illustration of Sezary cells
- Biography of Sezary (in French)
- Sezary Syndrome lymphoma information
- Doctor's doctor
- Cutaneous Lymphoma Foundation
- Clinical trial number NCT00106431 for "A Single Agent Phase II Study of Romidepsin (Depsipeptide, FK228) in the Treatment of Cutaneous T-cell Lymphoma (CTCL)" at ClinicalTrials.gov
- Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan
