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. © 2012 Royal College of Physicians of Edinburgh.