Treatment of lymphoblastic lymphomas in children in Russia: preliminary results of a multicenter retrospective study

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Abstract

Background. Lymphoblastic lymphomas (LBL) are an aggressive variant of non-Hodgkin’s lymphomas (NHL), which ranks second in frequency among non-Hodgkin’s lymphomas in children. According to international data, the effectiveness of LBL therapy in children reaches 85 %. In Russia, information on the treatment results for this disease is extremely limited.

Aim. To present preliminary data on the results of LBL therapy in children in Russia.

Materials and methods. The study included 392 pediatric LBL patients, treated in 57 specialized hospitals of the Russian Federation from 1999 to 2024.

Results. The overall and event-free survival rates were 83 % (95 % confidence interval 78–88) and 70 % (95 % confidence interval 64–78), respectively. In addition, the analysis revealed a high frequency of thrombotic complications (29 % of all patients), especially in cases of T-cell LBL. No significant differences in disease outcomes were found between tumor immunophenotypes, the type of asparaginase, and the stage of the disease. A separate analysis of the results of LBL therapy according to the ALL IC-BFM 2002 / 2009 protocols (146 patients) with stricter criteria for the response to induction therapy (tumor reduction of more than 70 %) and early chemotherapy intensification with high-dose blocks at the N. N. Blokhin National Medical Research Center of Oncology showed the event-free and overall survival of about 90 %. The Cox regression model of the dependence of therapy outcomes on residual tumor volume at the end of induction in the main cohort of patients did not reveal an association between residual tumor volume and overall survival (p = 0.2), but there was a statistical association with event-free survival (p = 0.006).

Discussion. This paper presents preliminary data from the first multicenter study of LBL therapy in pediatric patients in the Russian Federation. According to our data, LBL survival rates in the Russia should be improved. An important problem in the treatment of LBL in children is therapy complications, especially the high incidence of thrombosis. In this regard, anticoagulant prophylaxis is recommended for patients with LBL, especially with the T-cell phenotype and the presence of risk factors. Further studies are also needed to identify high-risk patients for the development of new personalized therapy approaches.

Conclusion. The paper presents preliminary results from the first multicenter clinical study of LBL therapy in children in Russia, indicating the necessity of treatment approaches improvement for increase a treatment effectiveness.

About the authors

E. V. Volchkov

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Botkin Hospital, Moscow Healthcare Department

Author for correspondence.
Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-2574-1636
Russian Federation, 1 Samory Mashela St., Moscow 117198; ; 5 2nd Botkinskiy Proezd, Moscow 125284

T. Yu. Pavlova

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Email: md.pavlovaty@mail.ru
ORCID iD: 0000-0003-4999-5195
Russian Federation, 23 Kashirskoe Shosse, Moscow 115522

G. I. Shestopalov

Russian Children’s Clinical Hospital – branch of the N. I. Pirogov Russian National Research Medical University, Ministry of Health of Russia

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-2410-2867
Russian Federation, 117 Leninskiy Prospekt, Moscow 119571

L. S. Zubarovskaya

I. P. Pavlov First Saint Petersburg State Medical University, Ministry of Health of Russia

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0003-2594-7703

Raisa Gorbacheva Memorial Research Institute for Pediatric Oncology, Hematology and Transplantation

Russian Federation, 6–8 L’va Tolstogo St., Saint Petersburg 197022

A. V. Protsvetkina

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia

Email: a.procvetkina@dgoi.ru
Russian Federation, 1 Samory Mashela St., Moscow 117198

E. A. Burtsev

Morozov Children’s Municipal Clinical Hospital, Moscow Healthcare Department

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-3623-6547
Russian Federation, 1 / 9 4th Dobryninskiy Pereulok, Moscow 119049

D. S. Abramov

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0003-3664-2876
Russian Federation, 1 Samory Mashela St., Moscow 117198

G. R. Kazaryan

Nizhnevartovsk District Clinical Hospital

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-1881-7444
Russian Federation, 19 Marshala Zhukova St., Nizhnevartovsk 628616

V. V. Martynenko

Bryansk Regional Children’s Hospital

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0001-5203-7815
Russian Federation, 100 Stanke Dimitrova Prospekt, Bryansk 241050

K. A. Tsytsylina

Children’s Regional Clinical Hospital, Ministry of Health of Krasnodar Region

Email: volchcov.egor@yandex.ru
Russian Federation, 1 Ploshchad’ Pobedy, 350007 Krasnodar

V. O. Stepanova

Moscow Regional Oncology Dispensary

Email: v.o.stepanova@yandex.ru
Russian Federation, 6 Karbysheva St., Balashikha 143900

Yu. E. Belkina

Republican Children’s Clinical Hospital, Simferopol

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-4936-5719
Russian Federation, 71 Titova St, Simferopol 295034

V. A. Myasnikova

Chelyabinsk Regional Children’s Clinical Hospital

Email: volchcov.egor@yandex.ru
Russian Federation, 42A Blukhera St., Chelyabinsk 454087

N. A. Danilyuk

Regional Oncology Clinical Dispensary, Penza

Email: volchcov.egor@yandex.ru
Russian Federation, 37A Stroiteley Prospekt, Penza 440071

V. E. Tulyavko

RUDN University

Email: volchcov.egor@yandex.ru

Research Institute of Molecular and Cellular Medicine

Russian Federation, 8 Podolskoe Shosse, Moscow 115093

I. Y. Oleynik

Tomsk Regional Clinical Hospital

Email: volchcov.egor@yandex.ru
Russian Federation, 96 I. Chernykh St., Tomsk 634063

D. K. Polosukhina

Volgograd Regional Clinical Oncology Dispensary

Email: volchcov.egor@yandex.ru
Russian Federation, 78 Zemlyachki St., Volgograd 400117

I. A. Loskutov

Regional Children’s Clinical Hospital No. 1, Vladivostok

Email: volchcov.egor@yandex.ru
Russian Federation, 27 Ostryakova Prospekt, Vladivostok 690078

O. P. Oleshchuk

A.K. Piotrovich Children’s Regional Clinical Hospital

Email: volchcov.egor@yandex.ru
Russian Federation, 6 Progressivnaya St., Khabarovsk 680003

R. O. Panko

Samara Regional Children’s Clinical Hospital named after N. N. Ivanova

Email: volchcov.egor@yandex.ru
Russian Federation, 165A Karla Marksa Prospekt, Samara 443079

G. V. Bykova

Children’s Regional Clinical Hospital, Stavropol

Email: volchcov.egor@yandex.ru
Russian Federation, 3 Semashko St., Stavropol 355029

O. P. Tolmacheva

Irkutsk State Regional Children’s Clinical Hospital

Email: volchcov.egor@yandex.ru
Russian Federation, 4 Gagarina Boulevard, Irkutsk 664003

D. N. Koroleva

Republican Children’s Clinical Hospital, Ministry of Health of Republic of Udmurtia

Email: volchcov.egor@yandex.ru
Russian Federation, 79 Lenina St., Izhevsk 426009

S. V. Mkrtchyan

Ulyanovsk Regional Children’s Clinical Hospital named after political and public figure Yu.F. Goryachev

Email: volchcov.egor@yandex.ru
Russian Federation, 42 Radishcheva St., Ulyanovsk 432011

M. S. Yudakova

Regional Children’s Clinical Hospital named after N. V. Dmitrieva

Email: volchcov.egor@yandex.ru
Russian Federation, 1z Internatsionalnaya St., Ryazan 390039

M. B. Belogurova

Saint-Petersburg Clinical Scientific and Practical Center for Specialized Types of Medical Care (Oncology) named after N. P. Napalkov

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-7471-7181
Russian Federation, 68A Leningradskaya St., Saint Petersburg 197758

T. T. Valiev

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia; I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University)

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-1469-2365
Russian Federation, 23 Kashirskoe Shosse, Moscow 115522; Build. 2, 8 Trubetskaya St., Moscow 119048

N. V. Myakova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia

Email: volchcov.egor@yandex.ru
ORCID iD: 0000-0002-4779-1896
Russian Federation, 1 Samory Mashela St., Moscow 117198

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