What is the prevalence of Sézary syndrome in cutaneous T-cell lymphoma (CTCL)?

Updated: Apr 20, 2020
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Sézary syndrome accounts for about 5% of all cases of mycosis fungoides. The patient with Sézary syndrome has generalized exfoliative dermatitis or erythroderma and lymphadenopathy, as well as atypical T lymphocytes with cerebriform nuclei (more than 1000 per mm3) circulating in the peripheral blood or other evidence of a significant malignant T-cell clone in the blood, such as clonal T-cell gene rearrangement identical to that found in the skin. (See the images below.)

Erythroderma of Sézary syndrome. Erythroderma of Sézary syndrome.
Nail changes of Sézary syndrome. Nail changes of Sézary syndrome.

The T-cell gene rearrangement is demonstrated by molecular or cytogenetic techniques and/or an expansion of cells with a malignant T-cell immunophenotype (an increase of CD4+ cells such that the CD4/CD8 ratio is >10, and/or an expansion of T cells with a loss of one or more of the normal T-cell antigens [eg, CD2, CD3, CD5]). The circulating malignant cells tend to be CD7 and CD26 negative.

Although Sézary syndrome may be part of a continuum from erythrodermic mycosis fungoides, the WHO-EORTC classification for cutaneous lymphoma considers its behavior "aggressive." [1]

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