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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
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Your Question

Is Sezary syndrome hereditary? My father had this and now I think my sister has it.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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.[1]

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

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.[2] 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