Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children31310.24287/1726-1708-2020-19-1-108-115ШКОЛА ИММУНОЛОГАRosay–Dorfman – like lymphadenopathy in a patient with Wiskott–Aldrich syndrome: diagnostic difficultiesЛимфаденопатия по типу болезни Розаи–Дорфмана у пациента с синдромом Вискотта–Олдрича: сложности диагностикиhttps://orcid.org/0000-0001-5347-7150ShvetsO. A.ШвецО. А.
Russian Federation

MD, pediatrician allergologist-immunologist, 

117997, Moscow, Samory Mashela st., 1

канд. мед. наук, врач – аллергологиммунолог,

117997, Москва, ГСП-7, ул. Саморы Машела, 1 

shv1808@rambler.ruhttps://orcid.org/0000-0003-3664-2876AbramovD. S.АбрамовД. С.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-7684-9188KhorevaA. L.ХореваА. Л.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-6148-7209PershinD. E.ПершинД. Е.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-2467-2840KievaA. M.КиеваА. М.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2057-2036PhonkinA. V.ПшонкинА. В.
Russian Federation

Moscow

Москва

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России280320201911081152803202028032020Copyright ©; 2020, «D. Rogachev NMRCPHOI»Copyright ©; 2020, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2020«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/313

Wiskott–Aldrich Syndrome (WAS) is a primary immunodeficiency (PID), characterized by varying severity of typical symptoms: thrombocytopenia, infections, immune dysregulation and cancer predisposition. Therefore any lymphoproliferative complications in WAS patients require lymph node biopsy. However, the interpretation of the histological picture is often complicated and requires the knowledge of the lymph node pathomorphology in patients with immunodeficiency. This article describes a rare combination of late diagnosis of WAS complicated by lymphadenopathy, which clinically and morphologically resembles Rosai–Dorfman disease (RDD). Parents gave their consent to use information about the child, including the fotos, in the article.

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.

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