nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2024-11-4-71-80nodgo-1099Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSИстинная полицитемия у детей: обзор литературыPolycythemia vera in children: a literature reviewhttps://orcid.org/0000-0001-6028-9860БогдановА. В.BogdanovA. V.

Алексей Владимирович Богданов, аспирант, врач-детский онколог стационара кратковременного лечения

117997, Москва, ул. Саморы Машела, 1

Graduate Student, Pediatric Oncologist of the Hospital for Short-Term Treatment

1 Samory Mashela St., Moscow, 117997

alexeivld@mail.ruhttps://orcid.org/0000-0002-2057-2036ПшонкинА. В.PshonkinA. V.

к.м.н., врач-гематолог, врач-детский онколог, заведующий стационаром кратковременного лечения

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Hematologist, Pediatric Oncologist, Head of the Hospital for Short-Term Treatment

1 Samory Mashela St., Moscow, 117997

alexey.pshonkin@gmail.comФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian Federation2024200120251147180Copyright © Богданов А.В., Пшонкин А.В., 20252025Богданов А.В., Пшонкин А.В.Bogdanov A.V., Pshonkin A.V.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/1099

Хронические миелопролиферативные заболевания (ХМПЗ) представляют собой клональные миелоидные заболевания, характеризующиеся гиперпродукцией дифференцированных гемопоэтических клеток. Истинная полицитемия (ИП) относится к группе Ph-негативных ХМПЗ и связана с высоким риском развития тромбоза, геморрагических осложнений и фенотипической трансформацией заболевания в миелофиброз или острый миелобластный лейкоз. ИП встречается крайне редко среди детей, в первую очередь при диагностике заболевания необходимо исключить вторичную причину эритроцитоза. Заболеваемость ИП составляет 0,18 на 100 тыс. детского населения в год. Только половина пациентов детского возраста с ИП имеют симптомы, связанные с полицитемией. У большинства детей ИП диагностируют случайно, когда в общем анализе крови отмечается повышение концентрации гемоглобина или показателя гематокрита. Мутация в гене JAK2 включена в диагностические критерии ИП, однако ее распространенность среди детей значительно ниже, чем среди взрослых. У взрослых пациентов с ИП определена тактика терапии в зависимости от риска возникновения осложнений: возраст старше 60 лет, тромботические осложнения в анамнезе. Дети крайне редко имеют в анамнезе тромботические события и поэтому относятся к группе низкого риска. В настоящее время для них не определены факторы риска, связанные с прогнозом и осложнениями заболевания, также ограничены данные о результатах применения циторедуктивной терапии, общего алгоритма по тактике лечения ИП в детской популяции не разработано, в основном опыт терапии экстраполируется с взрослых клинических протоколов лечения.

Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) are clonal myeloid diseases characterized by hyperproduction of differentiated hematopoietic cells. Polycythemia vera (PV) belongs to the group of MPNs and is associated with a high risk of thrombosis, hemorrhagic complications and phenotypic transformation of the disease into myelofibrosis or acute myeloid leukemia. PV is extremely rare among children, first of all, when diagnosing the disease, it is necessary to exclude the secondary cause of erythrocytosis. The incidence of PV is 0.18 per 100,000 people-years the child population. Only half of pediatric patients with PV have symptoms associated with polycythemia. In most children, PV is diagnosed accidentally when an increase in hemoglobin concentration or hematocrit is noted in the full blood count. The mutation JAK2 gene is included in the diagnostic criteria of PV, but its prevalence among children is significantly lower than among adults. In adult patients with PV, the tactics of therapy were determined depending on the risk of complications of the disease: age over 60 years, a history of thrombotic complications. Children rarely have a history of thrombotic events and therefore belong to the low-risk group. Risk factors related to the prognosis and complications of the disease have not been identified for them, data on the results of сytoreductive therapy are also limited, a general algorithm for the treatment of PV in the pediatric population has not been developed, mainly the experience of therapy is extrapolated from adult clinical treatment protocols.

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