RESUMEN
BACKGROUND: The new European Organization for Research and Treatment of Cancer classification considers Sézary syndrome (SS) among the aggressive epidermotropic cutaneous T-cell lymphomas (ECTLs). Recent technological advances have facilitated the diagnosis of this disease, but it remains practically incurable, with a median survival of about 2.5-5 years. Deaths are due in part to the iatrogenic effects of treatments, which suggests that the management of SS could be improved. OBJECTIVES: Retrospectively to study the prognostic criteria related to disease progression. METHODS: Thirty patients with SS were followed up in the Dermatology Department of the University Hospital in Nantes, France, between January 1989 and May 2000. The diagnosis of SS was based on at least three of the following criteria: erythroderma, histological evidence of ECTL, a level of 20% or more circulating Sézary cells, and loss of My7 antigen expression by basal cells of the epidermis. Two patients not seen again after the initial diagnosis were excluded from the statistical study. RESULTS: The median disease-specific survival of the 28 patients was 64.55 +/- 10.11 months. The prognostic factors found in univariate analysis were age at diagnosis (P = 0.0109), interval before diagnosis (P = 0.0566), lactate dehydrogenase (LDH) level (P = 0.042) and presence of the Epstein-Barr virus (EBV) genome (BHLF in in situ hybridization) in skin (P = 0.0079). The prognostic factors found in multivariate analysis were age, interval before diagnosis and presence of the EBV genome in keratinocytes. A decreased number of Langerhans cells in the epidermis did not appear to be a prognostic factor. CONCLUSIONS: Our study confirms the prognostic value of age and LDH level, and for the first time demonstrates the prognostic value of the identification of the EBV genome in the skin. This seems consistent with a marked immune deficit during severe forms of SS.