Sézary's disease

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Sézary disease
Classification and external resources
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).
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) (/ˌsˌzɑːˈr/) 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[edit]

Sézary cells: pleomorphic abnormal T cells with convoluted nucleus (Peripheral blood - MGG stain)

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:

  1. Generalized erythroderma[2]
  2. Lymphadenopathy[2]
  3. Atypical T-cells ("Sézary cells") in the peripheral blood[2]
  4. Hepatosplenomegaly[6]

Diagnosis[edit]

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[edit]

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[edit]

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[edit]

References[edit]

  1. ^ synd/3594 at Who Named It?
  2. ^ a b c d e f g h i j k l Cuneo A, Castoldi. "Mycosis fungoidses/Sezary's syndrome". Retrieved 2008-02-15. 
  3. ^ Thangavelu, Maya. "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Retrieved 2008-02-15. 
  4. ^ "Causes and Symptoms". Retrieved 2008-02-15. 
  5. ^ a b Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2. 
  6. ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15. 
  7. ^ a b c "Diagnosis". Retrieved 2008-02-15. 

External links[edit]