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Genetics Home Reference: your guide to understanding genetic conditions
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Sézary syndrome

Reviewed March 2013

What is Sézary syndrome?

Sézary syndrome is an aggressive form of a type of cancer called cutaneous T-cell lymphoma. Cutaneous T-cell lymphomas occur when certain immune cells, called T cells, become cancerous; these cancers characteristically affect the skin, causing different types of skin lesions. In Sézary syndrome, the cancerous T cells are called Sézary cells and are found in the skin, lymph nodes, and blood. A characteristic of Sézary cells is an abnormally shaped nucleus, described as cerebriform.

People with Sézary syndrome develop a red, severely itchy rash (erythroderma) that covers large portions of their body. Sézary cells are found in the rash. However, the skin cells themselves are not cancerous; the skin problems result when Sézary cells move from the blood into the skin. People with Sézary syndrome also have enlarged lymph nodes (lymphadenopathy). Other common signs and symptoms of this condition include hair loss (alopecia), thickened skin on the palms of the hands and soles of the feet (palmoplantar keratoderma), abnormalities of the fingernails and toenails, and lower eyelids that turn outward (ectropion). Some people with Sézary syndrome are less able to control their body temperature than people without the condition.

The cancerous T cells can spread to other organs in the body, including the lymph nodes, liver, spleen, and bone marrow. In addition, affected individuals have an increased risk of developing another lymphoma or other type of cancer.

Sézary syndrome occurs in adults over age 60 and progresses rapidly; historically, affected individuals survived an average of 2 to 4 years after development of the condition, although survival has improved with newer treatments.

Although Sézary syndrome is sometimes referred to as a variant of another cutaneous T-cell lymphoma called mycosis fungoides, these two cancers are generally considered separate conditions.

How common is Sézary syndrome?

Sézary syndrome is a rare condition, although its prevalence is unknown. It is the second most common form of cutaneous T-cell lymphoma after mycosis fungoides, accounting for approximately 3 to 5 percent of cases of cutaneous T-cell lymphoma.

What genes are related to Sézary syndrome?

The cause of Sézary syndrome is unknown. Most affected individuals have one or more chromosomal abnormalities, such as the loss or gain of genetic material. These abnormalities occur during a person's lifetime and are found only in the DNA of cancerous cells. Abnormalities have been found on most chromosomes, but some regions are more commonly affected than others. People with this condition tend to have losses of DNA from regions of chromosomes 10 and 17 or additions of DNA to regions of chromosomes 8 and 17. It is unclear whether these alterations play a role in Sézary syndrome, although the tendency to acquire chromosomal abnormalities (chromosomal instability) is a feature of many cancers. It can lead to genetic changes that allow cells to grow and divide uncontrollably.

How do people inherit Sézary syndrome?

The inheritance pattern of Sézary syndrome has not been determined. This condition occurs in people with no history of the disorder in their family and is not thought to be inherited in most cases.

Where can I find information about diagnosis or management of Sézary syndrome?

These resources address the diagnosis or management of Sézary syndrome and may include treatment providers.

  • Cancer Research UK: Treatments for Cutaneous T-Cell Lymphoma (http://www.cancerresearchuk.org/cancer-help/type/non-hodgkins-lymphoma/about/types/cutaneous-t-cell-lymphoma)
  • Cutaneous Lymphoma Foundation: Sezary Syndrome Fast Facts (http://www.clfoundation.org/sites/default/files/content/Fact%20Sheet_SS.pdf)
  • Genetic Testing Registry: Sezary syndrome (http://www.ncbi.nlm.nih.gov/gtr/conditions/C0036920)
  • Lymphoma Research Foundation: Cutaneous T-Cell Lymphoma Treatment Options (http://www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300151)
  • National Cancer Institute: Mycosis Fungoides and the Sézary Syndrome Treatment (http://www.cancer.gov/cancertopics/pdq/treatment/mycosisfungoides/Patient/page1)

You might also find information on the diagnosis or management of Sézary syndrome in Educational resources (http://www.ghr.nlm.nih.gov/condition/sezary-syndrome/show/Educational+resources) and Patient support (http://www.ghr.nlm.nih.gov/condition/sezary-syndrome/show/Patient+support).

General information about the diagnosis (http://ghr.nlm.nih.gov/handbook/consult/diagnosis) and management (http://ghr.nlm.nih.gov/handbook/consult/treatment) of genetic conditions is available in the Handbook. Read more about genetic testing (http://ghr.nlm.nih.gov/handbook/testing), particularly the difference between clinical tests and research tests (http://ghr.nlm.nih.gov/handbook/testing/researchtesting).

To locate a healthcare provider, see How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

Where can I find additional information about Sézary syndrome?

You may find the following resources about Sézary syndrome helpful. These materials are written for the general public.

You may also be interested in these resources, which are designed for healthcare professionals and researchers.

What other names do people use for Sézary syndrome?

  • Sezary erythroderma
  • Sezary's lymphoma
  • Sezary syndrome

For more information about naming genetic conditions, see the Genetics Home Reference Condition Naming Guidelines (http://ghr.nlm.nih.gov/ConditionNameGuide) and How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What if I still have specific questions about Sézary syndrome?

Ask the Genetic and Rare Diseases Information Center (http://rarediseases.info.nih.gov/GARD/).

What glossary definitions help with understanding Sézary syndrome?

alopecia ; bone marrow ; cancer ; cell ; cutaneous ; DNA ; erythroderma ; inheritance ; inheritance pattern ; inherited ; keratoderma ; lymph ; lymphoma ; nucleus ; palmoplantar keratoderma ; prevalence ; syndrome

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://www.ghr.nlm.nih.gov/glossary).

References

  • Campbell JJ, Clark RA, Watanabe R, Kupper TS. Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors. Blood. 2010 Aug 5;116(5):767-71. doi: 10.1182/blood-2009-11-251926. Epub 2010 May 18. (http://www.ncbi.nlm.nih.gov/pubmed/20484084?dopt=Abstract)
  • Caprini E, Cristofoletti C, Arcelli D, Fadda P, Citterich MH, Sampogna F, Magrelli A, Censi F, Torreri P, Frontani M, Scala E, Picchio MC, Temperani P, Monopoli A, Lombardo GA, Taruscio D, Narducci MG, Russo G. Identification of key regions and genes important in the pathogenesis of sezary syndrome by combining genomic and expression microarrays. Cancer Res. 2009 Nov 1;69(21):8438-46. doi: 10.1158/0008-5472.CAN-09-2367. Epub 2009 Oct 20. (http://www.ncbi.nlm.nih.gov/pubmed/19843862?dopt=Abstract)
  • Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008 Mar 15;371(9616):945-57. doi: 10.1016/S0140-6736(08)60420-1. Review. (http://www.ncbi.nlm.nih.gov/pubmed/18342689?dopt=Abstract)
  • Iżykowska K, Przybylski GK. Genetic alterations in Sezary syndrome. Leuk Lymphoma. 2011 May;52(5):745-53. doi: 10.3109/10428194.2010.551159. Epub 2011 Feb 16. Review. (http://www.ncbi.nlm.nih.gov/pubmed/21323514?dopt=Abstract)
  • Laharanne E, Oumouhou N, Bonnet F, Carlotti M, Gentil C, Chevret E, Jouary T, Longy M, Vergier B, Beylot-Barry M, Merlio JP. Genome-wide analysis of cutaneous T-cell lymphomas identifies three clinically relevant classes. J Invest Dermatol. 2010 Jun;130(6):1707-18. doi: 10.1038/jid.2010.8. Epub 2010 Feb 4. (http://www.ncbi.nlm.nih.gov/pubmed/20130593?dopt=Abstract)
  • Rosen ST, Querfeld C. Primary cutaneous T-cell lymphomas. Hematology Am Soc Hematol Educ Program. 2006:323-30, 513. Review. (http://www.ncbi.nlm.nih.gov/pubmed/17124079?dopt=Abstract)
  • van Doorn R, van Kester MS, Dijkman R, Vermeer MH, Mulder AA, Szuhai K, Knijnenburg J, Boer JM, Willemze R, Tensen CP. Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood. 2009 Jan 1;113(1):127-36. doi: 10.1182/blood-2008-04-153031. Epub 2008 Oct 1. (http://www.ncbi.nlm.nih.gov/pubmed/18832135?dopt=Abstract)
  • Wong HK, Mishra A, Hake T, Porcu P. Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome). Br J Haematol. 2011 Oct;155(2):150-66. doi: 10.1111/j.1365-2141.2011.08852.x. Epub 2011 Aug 25. Review. (http://www.ncbi.nlm.nih.gov/pubmed/21883142?dopt=Abstract)
  • Yamashita T, Abbade LP, Marques ME, Marques SA. Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update. An Bras Dermatol. 2012 Nov-Dec;87(6):817-28; quiz 829-30. Review. (http://www.ncbi.nlm.nih.gov/pubmed/23197199?dopt=Abstract)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: March 2013
Published: December 22, 2014