Therapy of
lymphoblastic lymphoma (LL) has evolved with use of
chemotherapy regimens modeled after those for
acute lymphocytic leukemia (ALL). We treated 33 patients with LL with the intensive
chemotherapy regimens hyper-CVAD (fractionated
cyclophosphamide,
vincristine,
Adriamycin, and
dexamethasone) or modified hyper-CVAD used for ALL at our institution. Induction consolidation was administered with 8 or 9 alternating cycles of
chemotherapy over 5 to 6 months with intrathecal
chemotherapy prophylaxis, followed by maintenance
therapy. Consolidative
radiation therapy was given to patients with
mediastinal disease at presentation. No consolidation with autologous or allogeneic
stem cell transplantation was performed. At diagnosis, 80% were T-cell immunophenotype, 70% were stages III to IV, 70% had mediastinal involvement, and 9% had central nervous system (
CNS) disease. Of the patients, 30 (91%) achieved complete remission, and 3 (9%) achieved partial response. Within a median of 13 months, 10 patients (30%) relapsed or progressed. Estimates for 3-year progression-free and overall survival for the 33 patients were 66% and 70%, respectively. Estimates for the patients with known T-cell immunophenotype were 62% and 67%, respectively. No parameters (eg, age, stage, serum
lactate dehydrogenase [LDH],
beta(2) microglobulin) appeared to influence outcome except for
CNS disease at presentation. Modification of the hyper-CVAD regimen with
anthracycline intensification did not improve outcome. Other modifications of the program could include incorporation of
monoclonal antibodies and/or
nucleoside analogs, particularly for slow responders or those with residual
mediastinal disease.