Overall survival in erythrodermic cutaneous T-cell lymphoma: an analysis of prognostic factors in a cohort of patients with erythrodermic cutaneous T-cell lymphoma

Abstract

Background: The most common cutaneous T-cell lymphomas (CTCLs) are mycosis fungoides and Sézary syndrome.

Aim: To determine whether blood stage and other prognostic variables affect overall survival (OS) in CTCL.

Methods: We studied retrospectively 1197 CTCL patients seen at the M.D. Anderson Cancer Center since 1987.

Results: We identified 124 (10.3%) patients with erythrodermic CTCL (E-CTCL), 63% of whom had positive gene rearrangements in skin and 19 of whom had no evidence of hematologic involvement. The median age at diagnosis was 63 years (range, 26-90 years); the male to female ratio was 1.3 : 1. OS curves were estimated by the Kaplan-Meier method and compared using log-rank tests. The median OS in all 124 E-CTCL patients was 5.1 years (range, 0.4-18.6 years) regardless of the cause of death or blood involvement. Patients were stratified by the H0-H4 staging system with manual or flow cytometric determination of Sézary cell counts (Russell-Jones R, Whittaker SJ. Sézary syndrome: diagnostic criteria and therapeutic options. Semin Cutan Med Surg 2000; 19: 100-108). The median OS was 7.6 years for H0-H2 (< 1000 Sézary cells/L) (n = 23), 5.4 years for H3 (>or= 1000 to <or= 10,000 Sézary cells/L) (n = 79), and 2.4 years for H4 (>or= 10,000 Sézary cells/L) (n = 22) (P = 0.011). Treatment with systemic steroids, age, serum lactate dehydrogenase, and white blood cell count >or= 20,000 microL were significant prognostic factors, but large cell transformation, T-cell receptor gene rearrangement, tumor-node-metastasis stage, treatments, and CD4 : CD8 ratio were not. In multivariate analysis, advanced age and elevated lactate dehydrogenase were the strongest predictors of a poor prognosis.

Conclusions: Serum LDH and age were the strongest predictive factors for OS in E-CTCL.