Sézary syndrome: a study of 176 patients at Mayo Clinic.

BACKGROUND

Sézary syndrome (SS), a leukemic variant of cutaneous T-cell lymphoma, is characterized by erythroderma and by atypical lymphocytes (Sézary cells) in peripheral blood. Although numerous studies have examined the range of disease in cutaneous T-cell lymphoma, a relative paucity of data exists to describe the long-term outcome of patients with SS.

OBJECTIVE

We sought to study long-term survival and prognostic factors of patients with SS.

METHODS

A retrospective chart review was conducted to identify patients with SS seen at Mayo Clinic from 1976 to 2010. Cox proportional hazards regression models, adjusted for age, were fit to evaluate factors associated with overall survival.

RESULTS

In total, 176 patients were identified with a clinicopathologic diagnosis of SS. Overall survival was 86.1% and 42.3% at 1 and 5 years, respectively, after diagnosis (median survival, 4.0 years). After adjustment for age, potential predictors of worse survival included lactate dehydrogenase level at presentation (hazard ratio [HR] 1.71; 95% confidence interval [CI] 1.18-2.47 per doubling), prior diagnosis of mycosis fungoides (HR 2.68; 95% CI 1.44-4.98), and the presence of T-cell receptor gene rearrangements in skin (HR 2.59; 95% CI 1.38-4.87) and in blood (HR 2.05; 95% CI 1.00-4.21).

LIMITATIONS: This study is retrospective and represents a single academic center population.

CONCLUSIONS

To our knowledge, this research evaluated the largest population of patients with SS studied to date. It shows that overall survival continues to be poor, with a median survival of 4.0 years after diagnosis.