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 (≥ 1000 to ≤ 10,000 Sézary cells/L) (n = 79), and 2.4 years for H4 (≥ 10,000 Sézary cells/L) (n = 22) (P = 0.011). Treatment with systemic steroids, age, serum lactate dehydrogenase, and white blood cell count ≥ 20,000 µL 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.