Affiliation:
1. N.N. Blokhin National Medical Research Centre of Oncology of the Ministry of Health of Russia
2. Institute of Emergency and Reconstructive Surgery named after V.K. Gusak of the Ministry of Health of the Donetsk People’s Republic
3. Yoshkar-Ola Children’s City Hospital named after L.I. Sokolova
4. National Oncology Center of the Ministry of Health of the Republic of Azerbaijan
5. Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan
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.
Publisher
National Medical Research Center of Hematology of the Ministry of Health of the Russian Federation
Reference24 articles.
1. Kaseb H., Mukkamalla S.K.R., Rajasurya V. Anaplastic Large Cell Lymphoma. [Updated 2023 Jan 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. https://www.ncbi.nlm.nih.gov/books/NBK537150
2. Zhang X.R., Chien P.N., Nam S.Y., Heo C.Y. Anaplastic Large Cell Lymphoma: Molecular Pathogenesis and Treatment. Cancers (Basel). 2022; 14(7): 1650. DOI: 10.3390/cancers14071650.
3. Alaggio R., Amador C., Anagnostopoulos I., et al. The 5th edition of the World Health Organization Classifi cation of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022; 36(7): 1720–48. DOI: 10.1038/s41375-022-01620-2.
4. Sibon D., Fournier M., Brière J., et al. Long-term outcome of adults with systemic anaplastic large-cell lymphoma treated within the Groupe d’Etude des Lymphomes de l’Adulte trials. J Clin Oncol. 2012; 30(32): 3939–46. DOI: 10.1200/JCO.2012.42.2345.
5. Montes-Mojarro I.A., Steinhilber J., Bonzheim I., et al. The Pathological Spectrum of Systemic Anaplastic Large Cell Lymphoma (ALCL). Cancers (Basel). 2018; 10(4): 107. DOI: 10.3390/cancers10040107.