What are the diagnostic criteria for Sézary syndrome in cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

For a diagnosis of Sézary syndrome, one or more of the following criteria should be present [3] :

  • An absolute Sézary cell count of least 1000 cells/µL

  • Demonstration of immunophenotypic abnormalities (an expanded CD4+ T-cell population that results in a CD4/CD8 ratio of more than 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 [4]

Following a diagnostic algorithm for the evaluation and diagnosis of erythroderma due to cutaneous T-cell lymphoma, versus erythroderma resulting from "reactive" causes, may be helpful. [74]


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