Есть ли место заместительной терапии гемофилии А у детей в настоящем и будущем?
https://doi.org/10.21682/2311-1267-2022-9-3-56-64
Аннотация
В последние годы мы наблюдаем революцию в области лечения пациентов с гемофилией А (ГА). Появление новых методов нефакторной и развитие генной терапии ставят перед врачами закономерный вопрос: каковы перспективы классического лечения ГА с применением концентратов факторов? Особенно это касается пациентов с ингибиторной формой ГА (ИГА), для которых до настоящего времени единственной опцией по снижению частоты геморрагических проявлений являлось использование препаратов шунтирующего действия, не позволяющих добиться полного контроля кровотечений. Улучшение результатов лечения больных ИГА было возможно только при полной эрадикации ингибиторов. Наиболее эффективным и безопасным методом, позволяющим избавиться от ингибиторов, является проведение терапии индукции иммунной толерантности (ИИТ). С появлением эмицизумаба и по-настоящему фантастических результатов его применения у пациентов с ИГА все чаще перед врачами встает вопрос о необходимости проведения ИИТ. Особенно остра эта проблема у детей с ИГА.
В данном обзоре представлены основные сведения о современных достижениях в терапии ГА, а также определено место препаратов заместительной терапии в настоящем и будущем.
Об авторе
П. А. Жарков
ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева» Минздрава России
Россия
Россия
Павел Александрович Жарков, д.м.н., врач-педиатр, врач-гематолог консультативного отделения, заведующий отделом патологии гемостаза.
Web of Science ResearcherID: AAP-9203-2020
117997, Москва, ул. Саморы Машела, 1
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Рецензия
Для цитирования:
Жарков П.А. Есть ли место заместительной терапии гемофилии А у детей в настоящем и будущем? Российский журнал детской гематологии и онкологии (РЖДГиО). 2022;9(3):56-64. https://doi.org/10.21682/2311-1267-2022-9-3-56-64
For citation:
Zharkov P.A. Is there any place for replacement therapy of hemophilia A in children in present and future? Russian Journal of Pediatric Hematology and Oncology. 2022;9(3):56-64. (In Russ.) https://doi.org/10.21682/2311-1267-2022-9-3-56-64
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