Outcome of Adult Lymphoblastic Lymphoma Patients Treated in the Real Life According to the Gimema LAL1913 Protocol: A Campus ALL Multicenter Study

The introduction of pediatric-inspired chemotherapy protocols for acute lymphoblastic leukemia (ALL) in the setting of adult patients affected by lymphoblastic lymphoma (LL) has improved the results in terms of outcomes. Within the Campus ALL network, we retrospectively collected data on 50 LL-patients from 23 hematology centers in Italy treated according to the GIMEMA LAL1913 protocol, with the aim of confirming the ALL results and of testing the effectiveness of an approach based on higher cumulative doses of the non-myelosuppressive components such as steroids and pegylated asparaginase, in addition to earlier and more intensive central nervous system prophylaxis. After cycle 3 (high dose methotrexate and cytarabine, C3), the cumulative incidence (CI) of PET-based complete response (CR) was of 83% (median time to response from protocol start: 3.7 months; CI95%: 3.1–4.2). With a median follow-up of 27.5 months, the 2-year overall survival (OS), disease free survival (DFS) ed event free survival rates were of 77%, 76% and 69%, respectively. The 2-year CI of relapse was of 26%. Multivariable analysis identified ECOG ≥ 2 as an independent prognostic factor for OS (HR = 3.279; CI95% 1.035–10.389, p = 0.044) and DFS (HR = 13.00, ICI95%: 3.383–57.909, p < 0.001), respectively. No significant impact was observed for pegylated asparaginase dose (reduced and/or omitted versus (vs.) full doses), age (≥ 55 vs. < 55), stage (0–I vs. II–IV), CD1a (negativity vs. other than CD1a negativity), bone marrow involvement (absent vs. ≤ 20%), or allotransplantation. The results of this study show the appropriateness of the LAL1913 protocol for the management of LL patients. Survival outcomes reflect those expected, although this drug combination was never tested before in a real-life setting. Taken together, these results suggest that LAL1913 might be considered as a reference protocol for the frontline treatment of adult LL patients.