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Аутоиммунный лимфопролиферативный синдром (обзор литературы)

https://doi.org/10.24287/1726-1708-2016-15-1-34-40

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Аннотация

Программированная клеточная гибель (апоптоз) лимфоцитов - неотъемлемое звено иммунного гомеостаза. Нарушение данного процесса может приводить к развитию аутоиммунного лимфопролиферативного синдрома (АЛПС) - уникального генетически обусловленного клинического состояния, при котором нарушения апоптоза лимфоцитов сочетаются с лимфопролиферацией и аутоиммунными проявлениями. С тех пор, как в XX веке заболевание было впервые описано, произошли дополнения в диагностике и в возможностях лечения этого синдрома. Наследуемый генетический дефект у большинства пациентов с АЛПС затрагивает FAS-сигнальный протеин, но с развитием молекулярно-генетической диагностики понятие неуточненного АЛПС постепенно размывается, обозначая новые мутации, расширяя спектр клинических проявлений и возраст дебюта заболевания. Современные терапевтические возможности позволяют с успехом контролировать течение заболевания.

Об авторах

Оксана Анатольевна Швец
Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России
Россия


Анна Юрьевна Щербина
Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России
Россия


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Для цитирования:


Швец О.А., Щербина А.Ю. Аутоиммунный лимфопролиферативный синдром (обзор литературы). Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2016;15(1):34-40. https://doi.org/10.24287/1726-1708-2016-15-1-34-40

For citation:


Shvets O.A., Shcherbina A.Y. Autoimmune lymphoproliferative syndrome (Review of literature). Pediatric Hematology/Oncology and Immunopathology. 2016;15(1):34-40. (In Russ.) https://doi.org/10.24287/1726-1708-2016-15-1-34-40

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ISSN 1726-1708 (Print)
ISSN 2414-9314 (Online)