Sezary syndrome (SS) is a cutaneous T-cell lymphoma (CTCL) with distinct clinical features (erythroderma, palmoplantar hyperkeratosis, lymphadenopathy) and leukemic spread of neoplastic T-cells in peripheral blood (1). The term "Sezary syndrome'' was proposed in 1961 by Taswell and Winkelmann (2). In 1938, Sezary and Bouvrain had described for the first time mononuclear cells with hyperconvoluted atypical nuclei in the skin and peripheral blood, for which they used the term "monster cells'' (3).
Clinical features Adult/elderly patients
Erythroderma, palmoplantar hyperkeratosis, alopecia Pruritus
Often unspecific with perivascular or band-like lymphocytic infiltrate Variable epidermotropism of atypical lymphocytes Admixture of eosinophils and plasma cells Immunophenotype
CD2+, CD3+, CD5+, CD4+, CD8-, CD30-, CD45RO+ Molecular biology
Clonal TCR rearrangement in majority of cases
Was this article helpful?