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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).
Classification and external resources
Specialty Oncology
ICD-10 C84.1
ICD-9 202.2
ICD-O M9701/3
DiseasesDB 8595
eMedicine med/1541 derm/566 med/3486
NCI Sézary disease
Patient UK Sézary disease
MeSH D012751

Sézary disease (also known as Sézary's disease or Sézary('s) 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 disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[2][3] There are currently no known causes of Sézary disease.[4]

Signs and symptoms

Sézary cell: pleomorphic abnormal T cell 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]


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]


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]


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


  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. 
  8. ^ Jawed, SI; Myskowski, PL; Horwitz, S; Moskowitz, A; Querfeld, C (February 2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.". Journal of the American Academy of Dermatology 70 (2): 223.e1–17; quiz 240–2. PMID 24438970. doi:10.1016/j.jaad.2013.08.033. 

External links