Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children44610.24287/1726-1708-2020-19-4suppl-50-61MATERIALS OF THE 3rd WORKING MEETING OF ALLERGISTS-IMMUNOLOGISTSМАТЕРИАЛЫ III РАБОЧЕГО СОВЕЩАНИЯ АЛЛЕРГОЛОГОВ-ИММУНОЛОГОВNon-infectious complications in the group of pediatric patients with chronic granulomatous diseaseХарактеристика неинфекционных проявлений хронической гранулематозной болезни в группе педиатрических пациентовhttps://orcid.org/0000-0001-9078-8206YukhachevaD. V.ЮхачеваД. В.
Russian Federation

an allergist-immunologist at the Department of Immunology

1 Samory Mashela St., Moscow 117997, Russia

врач аллерголог-иммунолог отделения иммунологии

117997, Москва, ул. Саморы Машела, 1

yukhachevadv@gmail.comhttps://orcid.org/0000-0001-9857-4456RodinaYu. A.РодинаЮ. А.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-2354-2588LaberkoA. L.ЛаберкоА. Л.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0001-5132-1267RoppeltA. A.РоппельтА. А.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0003-1267-9957BurlakovV. I.БурлаковВ. И.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-9083-4783DeripapaE. V.ДерипапаЕ. В.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0003-3564-6496KanN. Yu.КанН. Ю.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

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

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0003-3664-2876AbramovD. S.АбрамовД. С.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0001-7732-8184KonovalovD. M.КоноваловД. М.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-4056-7293PodlipaevaS. G.ПодлипаеваС. Г.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-7779-1969ZakharovI. V.ЗахаровИ. В.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-9639-2779KulakovskayaE. A.КулаковскаяЕ. А.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

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

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-0501-8686VarlamovaT. V.ВарламоваТ. В.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-7634-2053RaykinaE. V.РайкинаЕ. В.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0001-7317-7104TereshchenkoG. V.ТерещенкоГ. В.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-3113-4939ShcherbinaA. Yu.ЩербинаА. Ю.
Russian Federation

1 Samory Mashela St., Moscow 117997, Russia

117997, Москва, ул. Саморы Машела, 1

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России11012021194supplementприложение50610801202108012021Copyright ©; 2021, «D. Rogachev NMRCPHOI»Copyright ©; 2021, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2021«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/446

Chronic granulomatous disease (CGD) is a primary immunodeficiency (PID), characterized by a defective production of reactive oxygen species by phagocytes. Infectious diseases are a classic manifestation of this form of PID; however, many patients present with a variety of inflammatory and immune non-infectious complications. We analyzed non-infectious complications in a group of 60 patients with CGD, who were treated in Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology (Moscow, Russia) since 2012 to February 2020. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Non-infectious manifestations were recorded in 53/60 patients, the most frequent of which were granulomatous complications (81.7% (49/60) of cases) in the following organs - the gastrointestinal tract, lungs, liver and lymph nodes. The median age of granulomas presentation was 3 years, and 45.8% of patients had a combined granulomatous lesion of several organs. Hepatomegaly (40%), splenomegaly (31.7%), dermatitis (21.7%) and chorioretinal lesions (11.3%) were other non-infectious complications. Hematological features outside acute infectious episodes included monocytosis (26.7%), eosinophilia (20%), neutrophilia (13.3%). 96.7% of patients had anemia with a decrease level of serum iron (65%), 31.7% - with signs of hemolysis. Autoimmune manifestations included arthritis, thyroiditis, immune thrombocytopenia or immune neutropenia. Non-infectious complications comprise a significant part of the CGD manifestations and often are the first symptoms of the disease. Awareness of this fact is very important for multidisciplinary approach in treatment of CGD. Delayed diagnosis and immunosuppressive therapy of non-infectious complications without prophylactic antimicrobial therapy can be life-threatening for patients with CGD.

Хроническая гранулематозная болезнь (ХГБ) – это первичный иммунодефицит (ПИД), который относится к дефектам фагоцитарного звена и характеризуется нарушением продукции фагоцитами активных радикалов кислорода. Инфекционные заболевания являются классическим проявлением данной формы ПИД, тем не менее у многих пациентов наблюдаются различные воспалительные и иммунные неинфекционные осложнения. Нами проведен анализ неинфекционных проявлений у 60 пациентов с диагнозом ХГБ, которые наблюдались в НМИЦ ДГОИ им. Дмитрия Рогачева с февраля 2012 г. по май 2020 г. Исследование одобрено локальным этическим комитетом и утверждено решением ученого совета НМИЦ ДГОИ им. Дмитрия Рогачева. У 53/60 пациентов в исследуемой группе зарегистрированы неинфекционные проявления, наиболее частыми из которых были гранулематозные осложнения (81,7% (49/60) случаев) с поражением следующих органов: желудочно-кишечный тракт, легкие, печень и лимфатические узлы. Медиана возраста появления первых гранулематозных очагов составила 3 года, при этом у 45,8% пациентов отмечалось сочетанное гранулематозное поражение нескольких органов. Другими неинфекционными проявлениями были гепатомегалия (45%), спленомегалия (36,7%), дерматиты (21,7%) и хориоретиниты (11,3%). Гематологические изменения, не связанные с  инфекционными эпизодами, включали моноцитоз (26,7%), эозинофилию (20%), нейтрофилию (13,3%). Анемия наблюдалась у 96,7% пациентов: у 65% – со снижением уровня сывороточного железа, а у 31,7% – с признаками гемолиза. Аутоиммунные проявления включали единичные случаи артрита, тиреоидита, иммунной тромбоцитопении или иммунной нейтропении. Неинфекционные осложнения составляют значительную часть проявлений ХГБ, в том числе в манифестации заболевания, что требует осведомленности детских врачей разных специальностей. Отсроченная  постановка диагноза с невозможностью своевременного назначения пожизненной профилактической противомикробной терапии совместно с иммуносупрессивной терапией может быть опасной для жизни пациентов.

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