Sézary syndrome—clinical and histopathologic features, differential diagnosis, and treatment

Sézary syndrome (SS) is a rare subtype of cutaneous T-cell lymphoma marked by erythroderma, circulating neoplastic T cells, and poor prognosis. Its low incidence has made the study of its etiology, immunologic/molecular pathways, and effective treatments difficult. Because histopathology may be nonspecific in SS, microscopic findings must be correlated with the clinical presentation and the results of blood evaluation in order to make the diagnosis. Treatments that preserve, rather than compromise, the immune system are preferred.