Sézary syndrome
| Sézary disease | |
|---|---|
| Synonyms | Sézary's disease, Sézary('s) syndrome |
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| The bright red rash of Sezary syndrome | |
| Pronunciation | /ˌseɪˌzɑːˈriː/ |
| Classification and external resources | |
| Specialty | oncology, dermatology |
| ICD-10 | C84.1 |
| ICD-9-CM | 202.2 |
| ICD-O | M9701/3 |
| DiseasesDB | 8595 |
| eMedicine | med/1541 derm/566 med/3486 |
| MeSH | D012751 |
Sézary disease is a type of cutaneous lymphoma that was first described by Albert Sézary. The affected cells are T-cells (so it is a T-cell lymphoma) that have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy. There are currently no known causes of Sézary disease.
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, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:
Those who have Sézary disease often present with skin lesions that do not heal with normal medication. 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. Finally, a biopsy of a skin lesion can be performed to rule out any other causes.
Romidepsin, Vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.Mogamulizumab has been approved in Japan and waiting FDA approval in the United States. There are dozens of clinical trials, with a few in Phase III. Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.Stanford University has been pioneering low-dose radiation (1/3 of the standard), followed by stem-cell transplantation without chemo, as a potential cure with promising results.
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