BACKGROUND: Erythrodermic cutaneous T-cell lymphomas (CTCL) including Sézary syndrome have been successfully treated with daily administration of chlorambucil and prednisone (Winkelmann regimen). OBJECTIVES: Our purpose was to determine the efficacy and safety of a low-dose pulse chemotherapy with chlorambucil and fluocortolone in this stage of the disease. Fluocortolone has the same potency as prednisone but lacks a mineralocorticoid effect. PATIENTS/METHODS: Thirteen patients with erythrodermic CTCL (stages III-IVb) were treated with chlorambucil and fluocortolone therapy (chlorambucil 10-12 mg day-1 for 3 days and fluocortolone, first day 75 mg, second day 50 mg and third day 25 mg) as primary therapy in an uncontrolled pilot study. Treatment was started with pulses every 2 weeks; subsequently, the intervals were prolonged according to the clinical status. Clinical outcome, side-effects and long-term survival were assessed. RESULTS: Seven patients achieved a complete remission and six had a partial response (improved significantly). The mean duration of remissions was 16.5 (median 12) months. The mean number of cycles necessary during the first year was one cycle every 5 weeks. No treatment-related severe side-effects occurred. The long-term follow-up (mean 31.5, median 27 months) showed that six patients remained in complete remission and three showed a stable partial remission. Four patients died, two of them from their lymphoma. CONCLUSIONS: We conclude that pulse chemotherapy with chlorambucil and fluocortolone is effective and safe in the treatment of erythrodermic CTCL and should be considered as an alternative to the classical Winkelmann treatment scheme.
