title: Sezary syndrome: diagnosis and management.
abstract: We describe a case of a delayed diagnosis of Sezary syndrome in a patient with longstanding generalised pruritus, erythroderma, alopecia and nail dystrophy. We highlight some of the difficulties in diagnosis despite the fact that all patients have a characteristic circulating malignant T-cell lymphocyte in peripheral blood. These cells with cerebriform nuclei should be sought in peripheral blood films and further evaluated using flow cytometry in all patients with an erythrodermatous presentation. We summarise current treatment options although, as with all rare diseases, there are few randomised controlled clinical trials to inform decisions on optimal therapy for each individual patient.
authors.affiliation: Beatson West of Scotland Cancer Centre, 1053 Great Western Road, Glasgow G12 0YN, UK. catherine.ogilvie@ggc.scot.nhs.uk
authors.forename: C
authors.initials: C
authors.lastname: Ogilvie
authors.forename: R
authors.initials: R
authors.lastname: Jackson
authors.forename: M
authors.initials: M
authors.lastname: Leach
authors.forename: P
authors.initials: P
authors.lastname: McKay
mesh_terms: D000368:Aged; D000505:Alopecia / Q000175:diagnosis; D002467:Cell Nucleus / Q000473:pathology; D003873:Dermatitis, Exfoliative / Q000175:diagnosis; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D009262:Nails / Q000473:pathology; D011537:Pruritus / Q000175:diagnosis; D012751:Sezary Syndrome / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D012871:Skin Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology