Definition

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).

S├ęzary syndrome

Clinical features Adult/elderly patients

Erythroderma, palmoplantar hyperkeratosis, alopecia Pruritus

Marked lymphoadenopathy Histological features

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

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