Favorable outcome with hyper-CVAD in lymphoblastic lymphoma (LL)

Abstract

6597 Background: Outcome with LL has improved with use of more intensive chemotherapy regimens designed for acute lymphoblastic leukemia (ALL). Methods: From April 1992 to November 2001, 33 patients (pts) with newly diagnosed LL were treated with standard (n=22) [Kantarjian et al, J Clin Oncol 15:547, 2000] or modified (n=11) [Thomas et al, Blood 102:880a, 2003] hyper-CVAD regimens. Courses of hyper-fractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone were alternated with methotrexate (MTX) and ara-C. The modified regimen inserted liposomal daunorubicin and ara-C for course 2. Mediastinal irradiation (XRT) was planned for bulky mediastinal disease. Intrathecal CNS prophylaxis alternated MTX and ara-C on days 2 and 7 of the first 3–4 courses. POMP (6-MP, vincristine, MTX, prednisone) maintenance was given for at least 2 years, interrupted by intensifications (MTX/asparaginase with or without hyper-CVAD depending on regimen). Results: Median age was 28 yrs (range, 17–59) and 82% were males. 70% had mediastinal disease and 30% had pericardial and/or pleural effusions. Two pts had superior vena cava syndrome and 3 had CNS disease. T-cell immunophenotype was present in 80%. Five (15%) pts had bone marrow involvement. XRT was given to 74% with mediastinal disease. Overall complete remission (CR) rate was 91% (rest with mediastinal disease not qualifying for CR). With median follow-up of 48 months (range, 8 to 110+ months), 23 (70%) pts remained alive without disease. Ten pts progressed within a median of 13 months (6 with standard, 4 with modified hyper-CVAD). Shorter progression-free survival (PFS) was seen in pts with CNS disease at presentation. Three-year PFS was 77% for standard hyper-CVAD and 62% for the modified regimen. Three pts were successfully salvaged with allogeneic SCT. Conclusions: Hyper-CVAD is a highly active regimen which improves outcome in LL compared with conventional ALL regimens. Anthracycline intensification does not appear to improve outcome compared with standard hyper-CVAD. Incorporation of newer agents such as Campath and nelarabine could be considered for slow responders or those with residual mediastinal disease. No significant financial relationships to disclose.