detinfДЕТСКИЕ ИНФЕКЦИИCHILDREN INFECTIONS2072-81072618-8139Association of Pediatricians and Infection Disease doctors10.22627/2072-8107-2026-25-1-53-59detinf-1147Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEW OF THE LITERATUREОсобенности эссенциальной тромбоцитемии у новорожденных и детей раннего возрастаFeatures of essential thrombocythemia in newborns and infantshttps://orcid.org/0009-0001-3388-5744АхвердиеваЛ. Дж.AkhverdievaL. D.

Ахвердиева Лейла Джабировна, студент 6 курса Института материнства и детства  

Москва 

 Moscow 

ahverdievaleyla@mail.ruhttps://orcid.org/0009-0003-8625-7199МущероваД. М.MuscherovaD. M.

Мущерова Диана Максимовна, клинический ординатор 1-го года обучения кафедры госпитальной педиатрии им. академика В.А. Таболина Института материнства и детства 

Москва 

 Moscow 

diana.muscherova@mail.ruhttps://orcid.org/0000-0001-7305-9036СаркисянЕ. А.SarkisyanH. A.

Саркисян Егине Альбертовна, к.м.н., доцент кафедры госпитальной педиатрии им. академика В.А. Таболина Института материнства и детства 

Москва 

 Moscow 

heghinesarg@gmail.comhttps://orcid.org/0000-0003-3018-4392ЧеркасоваС. В.CherkasovaS. V.

Черкасова Светлана Вячеславовна, к.м.н., доцент кафедры госпитальной педиатрии им. академика В.А. Таболина Института материнства и детства 

Москва 

 Moscow 

cherkasovasv@mail.ruhttps://orcid.org/0009-0009-4325-1903ДрагуноваМ. В.DragunovaM. V.

Драгунова Мария Валерьевна, студент 6 курса Института материнства и детства 

Москва 

 Moscow 

milaniy-Ostre2016@yandex.ruhttps://orcid.org/0000-0002-9567-6761ШумиловП. В.ShumilovP. V.

Шумилов Петр Валентинович, д.м.н., профессор, заведующий кафедрой госпитальной педиатрии им. академика В.А. Таболина Института материнства и детства  

Москва 

 Moscow 

peter_shumilov@mail.ruФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Минздрава России (Пироговский университет)РоссияPirogov Russian National Research Medical University (Pirogov University)Russian Federation2026270320262515359Copyright © Ахвердиева Л.Д., Мущерова Д.М., Саркисян Е.А., Черкасова С.В., Драгунова М.В., Шумилов П.В., 20262026Ахвердиева Л.Д., Мущерова Д.М., Саркисян Е.А., Черкасова С.В., Драгунова М.В., Шумилов П.В.Akhverdieva L.D., Muscherova D.M., Sarkisyan H.A., Cherkasova S.V., Dragunova M.V., Shumilov P.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://detinf.elpub.ru/jour/article/view/1147

Эссенциальная тромбоцитемия (МКБ-10: D47.3, OMIM: #187950) — редкое клональное миелопролиферативное заболевание, характеризующееся стойким повышением количества тромбоцитов. Клиническое течение у новорожденных и детей раннего возраста преимущественно бессимптомное или малосимптомное, с парадоксально низким риском тромботических осложнений на фоне высокой частоты микрососудистых и геморрагических нарушений, обусловленных приобретенным синдромом фон Виллебранда (von Willebrand syndrome). Диагностика заболевания сложна в связи с необходимостью исключения более распространенных в неонатальном периоде реактивного тромбоцитоза и его наследственных форм. Перспективным направлением в настоящий момент является разработка педиатрических диагностических критериев и комплексных генетических анализов для выявления новых драйверных мутаций. Лечение носит консервативный характер и часто приводит к благоприятному исходу. Настоящий обзор систематизирует современные данные мировой литературы об особенностях течении эссенциальной тромбоцитемии у новорождённых и детей раннего возраста, акцентируя внимание на ключевых отличиях от взрослой формы заболевания.

Essential thrombocythemia (ICD-10: D47.3, OMIM: #187950) is a rare clonal myeloproliferative disorder characterized by a persistent increase in platelet count. The clinical course in newborns and young children is predominantly asymptomatic or oligosymptomatic, with a paradoxically low risk of thrombotic complications against a background of a high frequency of microvascular and haemorrhagic disorders caused by acquired von Willebrand syndrome. The diagnosis of essential thrombocythemia is complicated by the need to exclude reactive thrombocytosis and its hereditary forms, which are more common in the neonatal period. A promising direction at the moment is the development of paediatric diagnostic criteria and comprehensive genetic analyses to identify new driver mutations. Treatment is conservative and often leads to a favourable outcome. This review systematises current data from the world literature on the characteristics of essential thrombocythemia in newborns and young children, focusing on key differences from the adult form of the disease.

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