Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Sezary Syndrome

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_5276-3

Definition

Sezary syndrome (SS), named after the French dermatologist Albert Sezary (1880–1956), is a variant of cutaneous T-cell lymphoma (CTCL), a malignancy of mature T-helper cells involving the skin and blood. It is defined by the presence of an exfoliative erythroderma (an inflammation of the skin with erythema and scales involving over 80 % of the body surface), lymphadenopathy, and evidence of neoplastic cells in the skin and blood. “Sezary” cells refer to enlarged mature CD4+ lymphocytes with hyperconvoluted nuclei. Historically, the presence of Sezary cells in the peripheral blood was a defining criterion for SS. However, an increased number of these cells can be found in several benign dermatologic conditions and is no longer the agreed-upon standard. The presence of a clonally expanded population of CD4+ cells resulting in an increased CD4/CD8 > 10 in the blood is considered a more accurate measure. Immunophenotypic abnormalities which support a diagnosis of SS include...

Keywords

Allogeneic Bone Marrow Allogeneic Bone Marrow Transplantation Dermatologic Condition Sezary Syndrome Nail Dystrophy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
This is a preview of subscription content, log in to check access.

References

  1. Trautinger F, Knobler R, Willemze R et al (2006) EORTC consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome. Eur J Cancer 42:1014–1030CrossRefPubMedGoogle Scholar
  2. Willemze R (2003) Cutaneous T-cell lymphoma. In: Bologna J, Jorizzo J, Rapini R (eds) Dermatology. Elsevier, Amsterdam, pp 1921–1961Google Scholar
  3. Willemze R, Jaffe ES, Burg G et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105:3768–3785CrossRefPubMedGoogle Scholar

See Also

  1. (2012) Exfoliative Erythroderma. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 1360. doi: 10.1007/978-3-642-16483-5_2057Google Scholar
  2. (2012) Extracorporeal Photochemotherapy. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 1366. doi: 10.1007/978-3-642-16483-5_2071Google Scholar
  3. (2012) Lichenification. In: Schwab M (ed) Encyclopedia of Cancer, 3rd edn. Springer Berlin Heidelberg, p 2034. doi: 10.1007/978-3-642-16483-5_3346Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of DermatologyColumbia College of Physicians and SurgeonsNew YorkUSA
  2. 2.Department of DermatologyMedical University of ViennaViennaAustria