Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children60010.24287/1726-1708-2022-21-1-156-172LITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫChronic myeloid leukemia in children: a rare and unique entityХронический миелолейкоз у детей: редкая и уникальная болезньhttps://orcid.org/0000-0002-2731-4531BoychenkoE. G.БойченкоЭ. Г.
Russian Federation

Elmira G. Boychenko, Dr. Med. Ski., Head of the Department of Oncohematology, Chief Pediatric Hematologist of Saint Petersburg Healthcare Committee

14A Avangardnaya St., Saint-Petersburg, 198205 

Бойченко Эльмира Госмановна, д-р мед. наук, заведующая отделением онкогематологии, главный внештатный детский специалист гематолог Комитета по здравоохранению Санкт-Петербурга

198205, Санкт-Петербург, ул. Авангардная, 14А

boychenko-elmira@yandex.ruSaint Petersburg Children’s City Multidisciplinary Clinical Specialized Center of High Medical TechnologiesСПб ГБУЗ «Детский городской многопрофильный клинический специализированный центр высоких медицинских технологий»300320222111561723003202230032022Copyright ©; 2022, «D. Rogachev NMRCPHOI»Copyright ©; 2022, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2022«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/600

Chronic myeloid leukemia (CML) rarely occurs in the first two decades of life, accounting for 2% to 3% of leukemias in children and adolescents. Because of a lack of robust clinical study evidence, management of CML in children is not standardized and often follows guidelines developed for adults. Children and young adults tend to have a more aggressive clinical presentation than older adults, and recent data indicate that some genetic differences exist in pediatric and adult CML. Because children with CML may receive tyrosine kinase inhibitor (TKI) therapy for many decades, and are exposed to TKIs during a period of active growth, the acute and long-term toxicities of this option should be carefully evaluated against the complications associated with lifelong use of TKIs. This review aims to outlines the morphological, genetic and immuno-phenotypical findings of pediatric CML, and to recommend a uniform approach for the diagnostic procedures to be applied and for standardized treatment.

Хронический миелолейкоз (ХМЛ) является редким для детского возраста заболеванием и составляет 2–3% лейкемий у детей до 15 лет. Вследствие низкой частоты ХМЛ и недостаточного количества достоверных данных, полученных в ходе клинических исследований, практические стандарты оказания помощи детям и подросткам с данным заболеванием, в отличие от взрослых, не разработаны. В некоторых странах дети с ХМЛ лечатся взрослыми гематологами. Многие детские онкогематологи следуют руководствам по лечению ХМЛ, предназначенным для взрослых. Тем не менее существуют четкие различия между ХМЛ у взрослых и детей как в отношении клинических проявлений и динамики заболевания, так и в плане биологии этого процесса, а также факторов организма-хозяина, которые должны приниматься во внимание при пожизненном лечении детского ХМЛ. Цель настоящего обзора – представить основные сведения об эпидемиологии, патофизиологии и клинико-лабораторных характеристиках ХМЛ и изложить современные подходы к диагностике и лечению этого заболевания у детей и подростков.

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