Fast Five Quiz: Sézary Syndrome

Lauren C. Pinter-Brown, MD

Disclosures

November 01, 2021

Sézary syndrome is currently defined by the International Society for Cutaneous Lymphomas as a distinctive erythrodermic cutaneous T-cell lymphoma (potentially lacking the diagnostic histologic features in the skin) with hematologic evidence of leukemic involvement. The skin involvement should cover > 80% body surface area.

One or more of the following criteria should also be present:

  • An absolute Sézary cell count ≥ 1000 cells/µL

  • Demonstration of immunophenotypic abnormalities (an expanded CD4+ T-cell population that results in a CD4/CD8 ratio > 10; loss of any or all of the T-cell antigens CD2, CD3, CD4, and CD5; or loss of both CD4 and CD5

  • Demonstration of a T-cell clone in the peripheral blood by molecular or cytogenetic methods; flow cytometry may be useful for the differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas

Learn more about diagnosis of Sézary syndrome.

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