In Sézary syndrome, the cancerous T cells, called Sézary cells, are present in the blood, skin, and lymph nodes. A characteristic of Sézary ...YouAreHere:Home→Genetics→GeneticConditions→SézarysyndromeURLofthispage:https://medlineplus.gov/genetics/condition/sezary-syndrome/SézarysyndromeFromGeneticsHomeReference.LearnmoreDescriptionSézarysyndromeisanaggressiveformofatypeofbloodcancercalledcutaneousT-celllymphoma.CutaneousT-celllymphomasoccurwhencertainwhitebloodcells,calledTcells,becomecancerous;thesecancerscharacteristicallyaffecttheskin,causingdifferenttypesofskinlesions.InSézarysyndrome,thecancerousTcells,calledSézarycells,arepresentintheblood,skin,andlymphnodes.AcharacteristicofSézarycellsisanabnormallyshapednucleus,describedascerebriform.PeoplewithSézarysyndromedevelopared,severelyitchyrash(erythroderma)thatcoverslargeportionsoftheirbody.Sézarycellsarefoundintherash.However,theskincellsthemselvesarenotcancerous;theskinproblemsresultwhenSézarycellsmovefromthebloodintotheskin.PeoplewithSézarysyndromealsohaveenlargedlymphnodes(lymphadenopathy).Othercommonsignsandsymptomsofthisconditionincludehairloss(alopecia),skinswelling(edema),thickenedskinonthepalmsofthehandsandsolesofthefeet(palmoplantarkeratoderma),abnormalitiesofthefingernailsandtoenails,andlowereyelidsthatturnoutward(ectropion).SomepeoplewithSézarysyndromearelessabletocontroltheirbodytemperaturethanpeoplewithoutthecondition.ThecancerousTcellscanspreadtootherorgansinthebody,includingthelymphnodes,liver,spleen,andbonemarrow.Inaddition,affectedindividualshaveanincreasedriskofdevelopinganotherlymphomaorothertypeofcancer.Sézarysyndromemostoftenoccursinadultsoverage60andusuallyprogressesrapidly;historically,affectedindividualssurvivedanaverageof2to4yearsafterdevelopmentofthecondition,althoughsurvivalhasimprovedwithnewertreatments.AlthoughSézarysyndromeissometimesreferredtoasavariantofanothercutaneousT-celllymphomacalledmycosisfungoides,thesetwocancersaregenerallyconsideredseparateconditions.FrequencySézarysyndrom