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ГЕМОФАГОЦИТАРНЫЙ ЛИМФОГИСТИОЦИТОЗ

Аннотация

Гемофагоцитарный лимфогистиоцитоз – редкое и зачастую фатальное расстройство регуляции иммунного ответа, в основе патогенеза которого лежат дефекты клеточной цитотоксичности и гиперпродукция провоспалительных цитокинов. Различают генетически детерминированные формы гемофагоцитарного лимфогистиоцитоза и вторичные гемофагоцитарные синдромы, ассоциированные с инфекциями, злокачественными новообразованиями, аутоиммунными заболеваниями и иммунодефицитами. В статье дается краткое описание патогенеза, клинических проявлений и подходов к терапии гемофагоцитарных синдромов.
Ключевые слова: гемофагоцитарный лимфогистиоцитоз, иммуносупрессия, циклоспорин А, этопозид, дексаметазон.
(Вопросы современной педиатрии. 2009; 8(3):66-75)

Об авторах

М.А. Масчан
Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии МЗ РФ Российская детская клиническая больница Росздрава
Россия


Г.А. Новичкова
Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии МЗ РФ
Россия


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Для цитирования:


Масчан М., Новичкова Г. ГЕМОФАГОЦИТАРНЫЙ ЛИМФОГИСТИОЦИТОЗ. Вопросы современной педиатрии. 2009;8(3):66-75.

For citation:


Maschan M., Novichkova G. HEMOPHAGOCYTAL LYMPHOHISTIOCYTOSIS. Current Pediatrics. 2009;8(3):66-75.

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