Sézary disease

Sézary disease

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-CM 202.2
ICD-O M9701/3
DiseasesDB 8595
eMedicine med/1541 derm/566 med/3486
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]

Signs and symptoms

Sézary cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (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

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.[9]

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

  1. ↑ Sézary's cell at Who Named It?
  2. 1 2 3 4 5 6 7 8 9 10 11 12 Cuneo A, Castoldi. "Mycosis fungoidses/Sezary's syndrome". Retrieved 2008-02-15.
  3. ↑ Thangavelu M, Finn WG, Yelavarthi KK, Roenigk Jr HH, Samuelson E, Peterson L, Kuzel TM, Rosen ST (May 1997). "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Blood 89 (9): 3371–7. PMID 9129044.
  4. ↑ "Causes and Symptoms". Retrieved 2008-02-15.
  5. ↑ 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. 1 2 Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
  7. 1 2 3 "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. doi:10.1016/j.jaad.2013.08.033. PMID 24438970.
  9. ↑ Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 41. ISBN 978-1-4051-1376-2.

External links

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