ALCL Protocol of the DOIG Research Institute 2003: results of treatment of anaplastic large cell lymphoma in children in a comparative multicenter clinical trial

Abstract

Introduction. Anaplastic large cell lymphoma (ALCL) is characterized by clinical, morphological and immunohistochemical heterogeneity. Both intensive block regimens and programs similar to those used in the treatment of acute lymphoblastic leukemia (ALL) are used in therapy. Taking into account the prognostically unfavorable effect of the expression of T-cell markers by tumor cells, a protocol ALCL NII DOIG 2003 was developed, which takes into account not only risk groups, but also immunophenotypic features of the tumor substrate.Aim. To evaluate the treatment effectiveness of children with ALCL according to the protocol ALCL NII DOIG 2003 in comparison with the standard protocol NHL-BFM 95.Materials and methods. The study included 100 patients with newly diagnosed ALCL who received treatment from 2000 to 2023 in 5 federal and regional pediatric oncohematology departments. The patients were divided into 2 groups depending on the protocol of therapy: I group — 48 patients treated with protocol ALCL NII DOIG 2003; II group — 52 patients treated according to the NHL-BFM 95 protocol. The basis of the protocol ALCL NII DOIG 2003 was made up of high-intensity block regimes similar to those used in the treatment of high-risk T-precursor ALL. A comparative assessment of the overall, event-free and relapse-free survival of patients was carried out depending on the therapeutic protocol using the SPSS 21.0 program. Results. The 10-year overall survival rate in patients from I group was 95.3 ± 3.3 %, II group — 82.0 ± 5.4 % (p = 0.037). 10- year event-free survival in I group was 95.3 ± 3.3 %, II — 68.6 ± 6.5 % (p = 0.001). 10-year relapse-free survival in I group of patients was 97.3 ± 2.7 %, while in the II group — 74.4 ± 6.4 % (p = 0.003).Conclusion. The obtained results indicate the high effi ciency of a differentiated, immuno-oriented approach to the treatment of ALCL with the ALCL NII DOIG 2003 protocol, making it possible to achieve signifi cantly higher survival rates of patients compared to the standard treatment protocol.