Sezary Syndrome

Definition

Sezary syndrome (SS), named after the French dermatologist Albert Sezary (1880–1956), is a variant of cutaneous T-cell lymphoma (CTCL), a malignancy of mature T-helper cells involving the skin and blood. It is defined by the presence of an exfoliative erythroderma (an inflammation of the skin with erythema and scales involving over 80 % of the body surface), lymphadenopathy, and evidence of neoplastic cells in the skin and blood. “Sezary” cells refer to enlarged mature CD4+ lymphocytes with hyperconvoluted nuclei. Historically, the presence of Sezary cells in the peripheral blood was a defining criterion for SS. However, an increased number of these cells can be found in several benign dermatologic conditions and is no longer the agreed-upon standard. The presence of a clonally expanded population of CD4+ cells resulting in an increased CD4/CD8 > 10 in the blood is considered a more accurate measure. Immunophenotypic abnormalities which support a diagnosis of SS include...