Non-infectious complications in the group of pediatric patients with chronic granulomatous disease

D. V. Yukhacheva; Юхачева Д. В.; Yu. A. Rodina; Родина Ю. А.; A. L. Laberko; Лаберко А. Л.; A. A. Roppelt; Роппельт А. А.; V. I. Burlakov; Бурлаков В. И.; E. V. Deripapa; Дерипапа Е. В.; N. Yu. Kan; Кан Н. Ю.; A. L. Khoreva; Хорева А. Л.; D. S. Abramov; Абрамов Д. С.; D. M. Konovalov; Коновалов Д. М.; S. G. Podlipaeva; Подлипаева С. Г.; I. V. Zakharov; Захаров И. В.; E. A. Kulakovskaya; Кулаковская Е. А.; D. E. Pershin; Першин Д. Е.; T. V. Varlamova; Варламова Т. В.; E. V. Raykina; Райкина Е. В.; G. V. Tereshchenko; Терещенко Г. В.; A. Yu. Shcherbina; Щербина А. Ю.

doi:10.24287/1726-1708-2020-19-4suppl-50-61

Non-infectious complications in the group of pediatric patients with chronic granulomatous disease

Authors: Yukhacheva D.V.¹, Rodina Y.A.¹, Laberko A.L.¹, Roppelt A.A.¹, Burlakov V.I.¹, Deripapa E.V.¹, Kan N.Y.¹, Khoreva A.L.¹, Abramov D.S.¹, Konovalov D.M.¹, Podlipaeva S.G.¹, Zakharov I.V.¹, Kulakovskaya E.A.¹, Pershin D.E.¹, Varlamova T.V.¹, Raykina E.V.¹, Tereshchenko G.V.¹, Shcherbina A.Y.¹
Affiliations:
1. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian Federation
Issue: Vol 19, No 4 (2020): supplement
Pages: 50-61
Section: MATERIALS OF THE 3rd WORKING MEETING OF ALLERGISTS-IMMUNOLOGISTS
Submitted: 08.01.2021
Accepted: 08.01.2021
Published: 08.01.2021
URL: https://hemoncim.com/jour/article/view/446
DOI: https://doi.org/10.24287/1726-1708-2020-19-4suppl-50-61
ID: 446

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Abstract

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.

Keywords

primary immunodeficiency, chronic granulomatous disease, non-infectious complications, granulomatous complications

About the authors

D. V. Yukhacheva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: yukhachevadv@gmail.com
ORCID iD: 0000-0001-9078-8206

an allergist-immunologist at the Department of Immunology

1 Samory Mashela St., Moscow 117997, Russia

Russian Federation

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