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Genetic and Rare Diseases Information Center (GARD)

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Sezary syndrome


Other Names for this Disease

  • Sézary syndrome
  • Sezary's lymphoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Sezary syndrome?

What causes Sezary syndrome?

Is Sezary syndrome inherited?

How might Sezary syndrome be treated?

What is Sezary syndrome?

Sezary syndrome is a type of cutaneous T-cell lymphoma (CTCL), which belongs to a larger group of disorders known as non-Hodgkin’s lymphomas. Sezary syndrome is characterized by a widespread red rash that may cover most of the body, the presence of specific malignant cells (Sezary cells) in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. The exact cause of cutaneous T-cell lymphomas is currently unknown. Treatment options vary depending on severity and signs and symptoms but may include topical chemotherapy, radiation therapy, photochemotherapy, use of retinoids, and chemotherapy.[1] The prognosis is generally poor with a median survival between 2 and 4 years.[2]
Last updated: 4/30/2012

What causes Sezary syndrome?

Although several possible underlying causes of Sezary syndrome have been proposed, the exact cause remains unknown. Current research suggests that abnormalities of DNA are the underlying basis for cells becoming malignant (cancerous) in cutaneous T-cell lymphomas and other cancers. Although in some cases a predisposition to cancer may be inherited, most cancers result from abnormal genetic changes that occur spontaneously, possibly due to various environmental factors.[1] Some researchers have suggested that skin-associated microbes such as  Staphylococcus aureus and Chlamydia spp may play a role in antigen stimulation of T-cells, but definitive data has been very limited. Others have made associations between Sezary syndrome and cytomegalovirus and Epstein-Barr virus. More research regarding the potential causes of Sezary syndrome is needed and is currently under way.[3]
Last updated: 4/30/2012

Is Sezary syndrome inherited?

To our knowledge, there have not been any reports of familial cases of Sezary syndrome (e.g. the condition occurring in more than one member of a family), and the exact cause of the condition is currently unknown.[4]

A closely related condtion, mycosis fungoides (MF), has been reported to occur in more than one family member in several families.[4] Sezary syndrome has been considered a "leukemic variant" of MF in the past.[1] However, although there are conflicting reports in the literature, they are now more commonly thought to be separate conditions.
Last updated: 2/27/2013

How might Sezary syndrome be treated?

Treatment options for patients with Sézary syndrome include:[5]

These types of treatments are aimed at producing remissions and improving symptoms. Treatment, therefore, is considered palliative for most patients. Survival in excess of 8 years is common. Patients with Sezary syndrome may benefit from participation in clinical trials evaluating new approaches to treatment.[5]

More details about treatment of Sezary syndrome can be accessed by clicking here.

Last updated: 4/30/2012

References
  1. Cutaneous T-Cell Lymphomas. NORD. January 3, 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1124/viewAbstract. Accessed 4/30/2012.
  2. Vanessa Ngan. Cutaneous T-cell lymphoma. DermNet NZ. June 29, 2011; http://www.dermnetnz.org/dermal-infiltrative/cutaneous-t-cell-lymphoma.html. Accessed 4/30/2012.
  3. Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. March 15, 2008; 371(9616):945-957.
  4. Hodak E. et al. Familial mycosis fungoides: report of 6 kindreds and a study of the HLA system. J Am Acad Dermatol. March 2005; 52(3 Pt 1):393-402.
  5. Mycosis Fungoides and Sezary Syndrome Treatment (PDQ) - Treatment Option Overview. National Cancer Institute (NCI). 2010; http://www.cancer.gov/cancertopics/pdq/treatment/mycosisfungoides/HealthProfessional/page4. Accessed 2/8/2011.


Other Names for this Disease
  • Sézary syndrome
  • Sezary's lymphoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.