Sézary disease, or Sézary syndrome is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. ... The affected T-cells known as Sézary's ...SézarydiseaseFromWikipedia,thefreeencyclopediaJumptonavigationJumptosearchMedicalconditionSézarydiseaseOthernamesSézary'sdisease,Sézary('s)syndromeThebrightredrashofSezarysyndromePronunciation/ˌseɪˌzɑːˈriː/SpecialtyOncology,dermatologySézarydisease,orSézarysyndrome[1]isatypeofcutaneousT-celllymphomathatwasfirstdescribedbyAlbertSézary.[2]TheaffectedT-cellsknownasSézary'scells,andalsoasLutznercells,havepathologicalquantitiesofmucopolysaccharides.Sézarydiseaseissometimesconsideredalatestageofmycosisfungoideswithlymphadenopathy.[3][4]Contents1Signsandsymptoms2Diagnosis3Treatment3.1Radiationtherapy3.2Chemotherapy4Epidemiology5Seealso6References7ExternallinksSignsandsymptoms[edit]Sézarycell:pleomorphicabnormalTcellwiththecharacteristiccerebriformnuclei(Peripheralblood-MGGstain).SézarydiseaseandmycosisfungoidesarecutaneousT-celllymphomashavingaprimarymanifestationintheskin.[5]Thedisease'soriginisaperipheralCD4+T-lymphocyte,[3]althoughrarerCD8+/CD4-caseshavebeenobserved.[3]Epidermotropism(lymphocytesresidingintheepidermis)[6]byneoplasticCD4+lymphocyteswiththeformationofPautrier'smicroabscessesisthehallmarksignofthedisease.Althoughtheconditioncanaffectpeopleofallages,itiscommonlydiagnosedinadultsoverage60.[7][3]Thedominantsignsandsymptomsofthediseaseare:Generalizederythroderma–rednessoftheskin[3]Lymphadenopathy–swollen,enlargedlymphnodes[3]AtypicalT-cells–malignantlymphocytesknownas"Sézarycells"seenintheperipheralbloodwithtypicalcerebriformnuclei(brain-shaped,convolutednuclei)[8][3]Hepatosplenomegaly–enlargedliverandspleen[9]Palmoplantarkeratoderma–thickeningofthepalmsofthehands,andsolesofthefeet[10][11]Diagnosis[edit]Sézarysyndromeina61-year-oldmanpresentingin1972withunrelentingitchinessofsixmonths’duration.OntherightishisperipheralbloodfilmstainedwithPeriodicAcid-Schiff(PAS)showinganeoplasticTcell(Sézarycell).Tho