Treatment of Angioimmunoblastic Lymphadenopathy (AILD)-Type T-Cell Lymphoma Using Prednisone with or without the COPBLAM/IMVP-16 Regimen

OBJECTIVE: To describe the clinical course of patients with angioimmunoblastic lymphadenopathy (AILD)-type lymphoma with a sequential treatment with prednisone and COPBLAM/IMVP-16. DESIGN: A multicenter, prospective, nonrandomized trial. SETTING: University medical centers and community hospitals. PATIENTS: Sixty-seven patients were registered, 28 were excluded, and 39 patients were evaluable for response (median age, 59 years; range, 25 to 82 years) (stages I and II, 10%; stages III and IV, 90%; B symptoms, 74%). MEASUREMENTS: Response, survival, and relapse. INTERVENTION: Patients initially received prednisone and no further treatment if a complete remission was achieved. Relapsing or refractory patients were treated with COPBLAM/IMVP-16. Patients with life-threatening tumor progression or extension received COPBLAM/IMVP-16 initially. Treatments were chosen in accordance with tumor extension and response to prednisone. Treatment modalities were not compared. RESULTS: Twenty-eight patients received primary prednisone, 18 received secondary prednisone, and 11 received primary chemotherapy. The complete response rates (with 95% CIs) were 29% (CI, 12% to 46%), 56% (CI, 33% to 79%), and 64% (CI, 36% to 92%), respectively. The median observation time of surviving patients was 28 months (range, 7 to 53). The median overall survival time was 15 months. The probabilities (with 95% CIs) of overall survival, event-free survival, and relapse at 36 months were 40.5% (CI, 24% to 56%), 32.3% (CI, 17% to 47%), and 34.6% (CI, 14% to 56%), respectively. At the time of evaluation, 22 of 39 patients had died, 7 of noninfectious complications and 14 of infections. CONCLUSIONS: Prednisone with or without COPBLAM/IMVP-16 treatment in AILD-type lymphoma leads to complete remissions in about half of the patients and in long-term, disease-free survival for one third.