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Sezary Syndrome : Introduction

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Sezary syndrome (SS) - Introduction: SS is a rare variant of cutaneous T-cell lymphoma and accounts for <5% of cases. It is characterized by exfoliative erythroderma, generalized lymphadenopathy, and the presence of Sezary cells (neoplastic T-cells with cerebriform nuclei) in the peripheral blood, skin, and lymph nodes.

SS is closely related to mycosis fungoides (MF) and has been defined by some as the leukemic form of MF; however, recent studies suggest that they are separate entities based on the cell of origin and prognosis. The cell of origin in MF and SS is mature skin-homing CD4+ T-cell and circulating central memory T-cell respectively.

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