Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children97110.24287/1726-1708-2025-24-1-78-87ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИOutcomes of treatment of childhood acute promyelocytic leukemia according to the APL 2003/2008 protocols in Russia and BelarusРезультаты лечения острого промиелоцитарного лейкоза у детей в Российской Федерации и Республике Беларусь по протоколам ОПЛ 2003/2008https://orcid.org/0000-0001-8384-5073AleinikovaO. V.АлейниковаО. В.
Russian Federation

Moscow, Minsk

Алейникова Ольга Витальевна - член-корр. НАН РБ, д-р мед. наук, профессор, главный научный консультант Управления по планированию и стратегическому развитию службы детской онкологии и гематологии ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России.

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-7918-680XSadovskayaM. N.СадовскаяМ. Н.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-7130-8596BaydildinaD. D.БайдильдинаД. Д.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-0889-6986PopovA. M.ПоповА. М.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-9634-5828ZerkalenkovaE. A.ЗеркаленковаЕ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2352-7716OlshanskayaYu. V.ОльшанскаяЮ. В.
Russian Federation

Moscow

Москва

RudkovaE. A.РудковаЕ. А.
Belarus

Minsk

Минск

https://orcid.org/0000-0001-8228-4876DubrovinaM. E.ДубровинаМ. Э.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-7578-9657VoroninK. A.ВоронинК. А.
Russian Federation

Moscow

Москва

SamochatovaE. V.СамочатоваЕ. В.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2322-5734NovichkovaG. A.НовичковаГ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-0016-6698MaschanA. A.МасчанА. А.
Russian Federation

Moscow

Москва

The Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииThe Republican Medical Research Center of Pediatric Oncology, Hematology and Immunology of Ministry of Health of the Republic of BelarusГУ «Республиканский научно-практический центр детской онкологии, гематологии и иммунологии» Минздрава Республики Беларусь0804202524178872703202527032025Copyright ©; 2025, «D. Rogachev NMRCPHOI»Copyright ©; 2025, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2025«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/971

This study presents the results of treatment of 339 patients with acute promyelocytic leukemia (APL) from 57 regions of the Russian Federation (RF) and the Republic of Belarus (RB) according to the 2003/2008 APL treatment protocols, from 2008 to 1 January 2024. Based on the data from the AML-MRD-2018 protocol, the proportion of APL among childhood acute myeloid leukemia (AML) cases in the RF was investigated. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. It was established that the APL cases comprised 26% of AML cases, and APL incidence rate varied across the regions of the RF. Our findings suggest that the incidence of APL in Russian children is higher than in children from Europe and the USA, but further epidemiological research is needed to confirm this. The 5-year overall (OS) and event-free (EFS) survival rates in the patients treated according to the 2003/2008 APL protocols were 82% (95% confidence interval (CI) 78–86) and 73% (95% CI 67–78), respectively. In the low-risk group (LRG) the five-year OS and EFS rates were statistically significantly higher than in the high-risk group (HRG), with 5-year OS reaching 93% (95% CI 89–97) in the LRG and 63% (95% CI 55–72) (p < 0.001) in the HRG, and EFS – 85% (95% CI 79–91) and 52% (95% CI 44–63) (p < 0.001), respectively. The risk of relapse was higher in the HRG: 15% (95% CI 9.1–25) in the HRG vs 8.7% (95% CI 4.9–15%) in the LRG (p < 0.045). The difference in the survival rates in the LRG and HRG was attributable to a higher mortality rate during remission induction in the HRG as compared to the LRG: 30.1% and 2.9%, respectively. Notably, 83.7% of deaths were caused by brain haemorrhage. Decreasing early death rate is key to improving APL treatment results in children in the RF and RB.

В работе представлены результаты терапии 339 пациентов с острым промиелоцитарным лейкозом (ОПЛ), получивших лечение в 57 регионах Российской Федерации (РФ) и Республике Беларусь (РБ) по протоколам ОПЛ 2003/2008 с 2008 г. по 1 января 2024 г. На основании протокола ОМЛ-MRD-2018 выполнена оценка доли ОПЛ в структуре острого миелоидного лейкоза (ОМЛ) у детей в РФ. Данное исследование одобрено независимым этическим комитетом и утверждено решением ученого совета ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России. Установлено, что доля ОПЛ в структуре ОМЛ составила 26%, причем встречаемость ОПЛ в различных регионах РФ неоднородна. Полученные результаты свидетельствуют о более высокой заболеваемости ОПЛ у детей в РФ, чем в Европе и США, однако это требует дальнейшего подтверждения в эпидемиологических исследованиях. Показатели 5-летней общей (ОВ) и бессобытийной (БСВ) выживаемости у пациентов, получивших лечение по протоколам ОПЛ 2003/2008, составили 82% (95% доверительный интервал (ДИ) 78–86) и 73% (95% ДИ 67–78) соответственно. В группе низкого риска (ГНР) 5-летняя ОВ и БСВ были статистически значимо выше, чем в группе высокого риска (ГВР), и составили: ОВ – 93% (95% ДИ 89–97) и 63% (95% ДИ 55–72) (p < 0,001) соответственно, БСВ – 85% (95% ДИ 79–91) и 52% (95% ДИ 44–63) (p < 0,001) соответственно. Риск рецидива был выше в ГВР: 15% (95% ДИ 9,1–25) против 8,7% (95% ДИ 4,9–15) в ГНР (p < 0,045). Различия в показателях выживаемости в ГНР и ГВР были обусловлены высокой смертностью в индукции ремиссии в ГВР по сравнению с ГНР – 30,1% и 2,9% соответственно, причем причиной 83,7% смертей было кровоизлияние в мозг. Снижение показателей ранней смерти является ключевым для улучшения результатов лечения ОПЛ у детей в РФ и РБ.

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