аутоиммунная нейтропения

Аутоиммунный лимфопролиферативный синдром в гематологической клинике: обзор литературы и собственное наблюдение

РЕДКИЕ И СЛОЖНЫЕ ГЕМАТОЛОГИЧЕСКИЕ СИНДРОМЫ , , , , , , ,

И.Б. Ковынев1, Т.И. Поспелова1, А.В. Мишенин2, И.Н. Нечунаева2, Р.В. Тарновский1

1 ГБОУ ВПО «Новосибирский государственный медицинский университет», Новосибирск, Российская Федерация

2 МБУЗ «Городская клиническая больница № 2», Новосибирск, Российская Федерация

РЕФЕРАТ

В статье представлены обзор литературы и описание редкого наблюдения аутоиммунного лимфопролиферативного синдрома (ALPS — autoimmune lymphoploliferative syndrome). Заболевание связано с блоком апоптоза иммунных клеток, вызванного мутацией генов Fas-протеинов. Клинически процесс характеризуется прогрессирующим генерализованным лимфопролиферативным синдромом при картине реактивной гиперплазии в биоптате лимфоузлов и иммунными 1–3 ростковыми цитопениями. Обсуждаются вопросы трансформации в лимфому или системную красную волчанку и современной тактики лечения.

Ключевые слова: аутоиммунный лимфопролиферативный синдром, цитопении, аутоиммунная гемолитическая анемия, аутоиммунная нейтропения, лимфаденопатия, лимфопролиферативные заболевания, иммунная тромбоцитопеническая пурпура, лимфома.

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