Лимфаденопатия по типу болезни Розаи–Дорфмана у пациента с синдромом Вискотта–Олдрича: сложности диагностики

Синдром Вискотта–Олдрича (СВО) – первичное иммунодефицитное состояние (ПИДС), характеризующееся типичными симптомами разной степени тяжести: тромбоцитопения, инфекция, иммунная дизрегуляция и склонность к онкологическим заболеваниям. В связи с этим при любом лимфопролиферативном состоянии при СВО необходима биопсия лимфоузлов. Однако трактовка гистологической картины требует знания особенностей патоморфологии лимфоузлов у пациентов с иммунодефицитом и нередко представляет сложности. В данной статье представлено клиническое наблюдение с описанием редкого сочетания поздно диагностированного СВО с лимфаденопатией, клинически и морфологически напоминающей болезнь Розаи–Дорфмана в контексте ПИДС. Родители дали согласие на использование информации, в том числе фото ребенка, в научных исследованиях и публикациях.

болезнь Розаи–Дорфмана, гистология, синдром Вискотта–Олдрича, лимфопролиферативный синдром

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