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Sézary disease

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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) () 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 1
  • Diagnosis 2
  • Treatment 3
  • Epidemiology 4
  • See also 5
  • References 6
  • External links 7

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.[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 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. ^ a b c d e f g h i j k l
  3. ^
  4. ^
  5. ^ a b
  6. ^ a b
  7. ^ a b c
  8. ^

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
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