References

nodgo

Российский журнал детской гематологии и онкологии (РЖДГиО)

Russian Journal of Pediatric Hematology and Oncology

2311-12672413-5496

LTD “Graphica”

10.21682/2311-1267-2025-12-2-82-89

nodgo-1152

Research Article

ОБЗОРЫ ЛИТЕРАТУРЫ

LITERATURE REVIEWS

Особенности диагностики и лечения эссенциальной тромбоцитемии у детей

The features of the diagnosis and treatment of essential thrombocythemia in children

https://orcid.org/0000-0001-8088-1749

Краличкин

П. В.

Kralichkin

P. V.

Павел Викторович Краличкин - врач-детский онколог стационара кратковременного лечения НМИЦ ДГОИ им. Дмитрия Рогачева.

117997, Москва, ул. Саморы Машела, 1

Pediatric Oncologist of the Hospital for Short-Term Treatment at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.

1 Samory Mashela St., Moscow, 117997

pavel.kralichkin@dgoi.ru

https://orcid.org/0000-0002-2057-2036

Пшонкин

А. В.

Pshonkin

A. V.

к.м.н., доцент ВАК, врач-гематолог, врач-детский онколог, заведующий стационаром кратковременного лечения НМИЦ ДГОИ им. Дмитрия Рогачева.

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Associate Professor of the Higher Attestation Commission, Hematologist, Pediatric Oncologist, Head of the Hospital for Short-Term Treatment at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.

1 Samory Mashela St., Moscow, 117997

Alexey.Pshonkin@dgoi.ru

https://orcid.org/0000-0003-4384-6754

Жарков

П. А.

Zharkov

P. A.

д.м.н., доцент ВАК, врач-педиатр, врач-гематолог консультативного отделения, заведующий отделом патологии гемостаза НМИЦ ДГОИ им. Дмитрия Рогачева, Web of Science ResearcherID: AAP-9203-2020.

117997, Москва, ул. Саморы Машела, 1

Dr. of Sci. (Med.), Associate Professor of the Higher Attestation Commission, Pediatrician, Hematologist Outpatient Consultative Unit, Head of the Hemostasis Pathology Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, Web of Science ResearcherID: AAP-9203-2020.

1 Samory Mashela St., Moscow, 117997

pavel.zharkov@dgoi.ru

https://orcid.org/0000-0003-4911-0553

Новичкова

Г. А.

Novichkova

G. A.

д.м.н., профессор, научный руководитель НМИЦ ДГОИ им. Дмитрия Рогачева, главный внештатный детский специалист гематолог-онколог Минздрава России.

117997, Москва, ул. Саморы Машела, 1

Dr. of Sci. (Med.), Professor, Scientific Director at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, Chief Freelance Pediatric Oncologist and Hematologist of the Ministry of Health of Russia.

1 Samory Mashela St., Moscow, 117997

Novichkova.Galina@dgoi.ru

ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian Federation

2025

01082025

1228289

2025

Краличкин П.В., Пшонкин А.В., Жарков П.А., Новичкова Г.А.

Kralichkin P.V., Pshonkin A.V., Zharkov P.A., Novichkova G.A.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://journal.nodgo.org/jour/article/view/1152

Эссенциальная тромбоцитемия (ЭТ) – это клональное Ph-негативное миелопролиферативное новообразование с неконтролируемой пролиферацией мегакариоцитов, характеризующееся повышенным числом крупных и гигантских мегакариоцитов в костном мозге, тромбоцитозом, а также высоким риском развития тромбозов и/или кровотечений. Клиническая картина при ЭТ у детей вариабельна: в отличие от взрослых, у которых на первый план выходят геморрагические и тромботические события, большинство пациентов детского возраста не имеет клинических проявлений, кроме изменений в гемограмме. Критерии, применяемые для диагностики, а также определения факторов риска у взрослых пациентов не применимы для пациентов детского возраста. Терапевтические рекомендации, используемые для взрослых пациентов с ЭТ также не могут однозначно применяться у пациентов детского возраста. Кроме того, генетический профиль ЭТ у детей радикально отличается от такового у взрослых, что выражается в различной частоте соматических драйверных мутаций и большем количестве трижды-негативных случаев.

Цель данной работы – продемонстрировать особенности клинико-лабораторной картины, течения и терапии ЭТ у детей и подростков.

Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm with uncontrolled production of megakaryocytes. It is characterized by the presence of large and giant megakaryocytes in the bone marrow, which leads to an increase in platelet count. This condition can cause both thrombosis and bleeding. In children, the clinical presentation of ET can vary. Unlike adults, who often experience hemorrhagic and thrombotic events, most pediatric patients do not have any symptoms. Instead, they may only have changes in their blood count. The diagnostic criteria and risk factors for ET in adults are not directly applicable to children. Similarly, the treatment recommendations for adults with ET cannot be directly applied to children. The genetic profile of ET in children also differs from that in adults, leading to differences in the frequency of specific driver mutations and the number of cases of the disease.

The aim of this study is to describe the clinical and laboratory features, course, and treatment of ET in children and adolescents.

эссенциальная тромбоцитемияпатогенезтерапия

essential thrombocythemiapathogenesistherapy

The study was performed without external funding

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The authors declare that there are no conflicts of interest present.