Sézary syndrome

    Article Contributors: 
    Sean Klepper M.D.
    Stephen Lyle, M.D., ...

    Clinical Features:

    • The leukemic phase of mycosis fungoides, with a worse prognosis than MF
    • Pruritic erythroderma with scaling and edema, palmoplantar hyperkeratosis, alopecia, onychodystrophy and lymphadenopathy
    • Advanced cases may show widespread infiltration of the face, leading to leonine facies.

    Histologic Features:

    • Usually a relatively nonspecific perivascular or band-like upper dermal infiltrate of small lymphocytes, which may or may not display atypia (e.g., cerebriform nuclei)
    • Epidermotropism is present in only 40% of cases.
    • The skin findings are indistinguishable from the patch or plaque stage of mycosis fungoides.
    • Lymph nodes may show only dermatopathic lymphadenopathy or may show frank involvement with effacement of nodal architecture by lymphocytes with cerebriform nuclei.
    • The immunophenotype is identical to that of mycosis fungoides (helper T-cell phenotype): CD2+, CD3+, CD4+, CD5+, CD8-, CD30-.
    • Diagnosis requires the demonstration of over 1000 circulating Sézary cells per cubic millimeter in the peripheral blood with a CD4:CD8 ratio of over 10 to 1.

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