Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Sezary Syndrome

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


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...


Allogeneic Bone Marrow Allogeneic Bone Marrow Transplantation Dermatologic Condition Sezary Syndrome Nail Dystrophy 
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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