Аутоиммунный лимфопролиферативный синдром (обзор литературы)

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.

аутоиммунный лимфопролиферативный синдром, апоптоз, ген FAS, дубль-негативные Т-клетки, критерии диагностики, дифференциальный диагноз, лечение, сиролимус, прогноз, autoimmune lymphoproliferative syndrome, apoptosis, FAS gene, double-negative T-cells, diagnostic criteria, differential diagnosis, treatment, sirolimus, prognosis

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