World Library  
Flag as Inappropriate
Email this Article

Sézary disease

Article Id: WHEBN0003037267
Reproduction Date:

Title: Sézary disease  
Author: World Heritage Encyclopedia
Language: English
Subject: Lymphoma, Lymphocytic leukemia, Diffuse infiltrative lymphocytosis syndrome, Monocytic leukemia, Plasmacytoma
Publisher: World Heritage Encyclopedia

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]


  • 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]


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. ^ 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
  • Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan
This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from Project Gutenberg are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.