Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia

A. A. Mukhina; Мухина А. А.; I. N. Abramova; Абрамова И. Н.; N. V. Kuzmenko; Кузьменко Н. Б.; Yu. A. Rodina; Родина Ю. А.; T. V. Latysheva; Латышева Т. В.; E. A. Latysheva; Латышева Е. А.; A. M. Kostinova; Костинова А. М.; N. I. Ilyina; Ильина Н. И.; A. S. Katrysheva; Катрышева А. С.; E. V. Sudarikova; Сударикова Е. В.; S. B. Zimin; Зимин С. Б.; A. A. Korsunskii; Корсунский А. А.; L. Yu. Barycheva; Барычева Л. Ю.; L. T. Kubanova; Кубанова Л. Т.; L. R. Kalmetieva; Кальметьева Л. Р.; D. D. Proligina; Пролыгина Д. Д.; G. A. Davletbaieva; Давлетбаева Г. А.; L. G. Shakirova; Шакирова Л. Г.; E. M. Gracheva; Грачева Е. М.; O. S. Selezneva; Селезнева О. С.; K. P. Kuzmicheva; Кузьмичева К. П.; O. A. Richkova; Рычкова О. А.; E. V. Demikhova; Демихова Е. В.; V. G. Demikhov; Демихов В. Г.; I. O. Frolova; Фролова И. О.; E. V. Tymofeeva; Тимофеева Е. В.; S. N. Duryagina; Дурягина С. Н.; A. V. Gorenkova; Горенькова А. В.; E. V. Vlasova; Власова Е. В.; O. M. Laba; Лаба О. М.; N. V. Shakhova; Шахова Н. В.; A. Yu. Kutlyanceva; Кутлянцева А. Ю.; G. A.` Novichkova; Новичкова Г. А.; A. G. Rumyantsev; Румянцев А. Г.; A. Yu. Shcherbina; Щербина А. Ю.

doi:10.24287/1726-1708-2020-19-4suppl-18-29

Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia

Authors: Mukhina A.A.¹, Abramova I.N.¹, Kuzmenko N.V.¹, Rodina Y.A.¹, Latysheva T.V.², Latysheva E.A.², Kostinova A.M.², Ilyina N.I.², Katrysheva A.S.³, Sudarikova E.V.³, Zimin S.B.³, Korsunskii A.A.³, Barycheva L.Y.⁴, Kubanova L.T.⁴, Kalmetieva L.R.⁵, Proligina D.D.⁵, Davletbaieva G.A.⁵, Shakirova L.G.⁵, Gracheva E.M.⁶, Selezneva O.S.⁶, Kuzmicheva K.P.⁷, Richkova O.A.⁷, Demikhova E.V.⁸, Demikhov V.G.⁸, Frolova I.O.⁸, Tymofeeva E.V.⁶, Duryagina S.N.⁶, Gorenkova A.V.⁶, Vlasova E.V.⁹, Laba O.M.¹⁰, Shakhova N.V.¹¹, Kutlyanceva A.Y.¹, Novichkova G.A.¹, Rumyantsev A.G.¹, Shcherbina A.Y.¹
Affiliations:
1. Dmitry Rogachev National Medical Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian Federation
2. National Research Center Institute of Immunology, Federal Biomedical Agency of Russia
3. Speransky Children’s Municipal Clinical hospital №9, Healthcare Department of Moscow
4. Stavropol State Medical University, Ministry of Healthcare of Russian Federation
5. Bashkortostan Republican Pediatric Hospital, Ministry of Healthcare of Bashkortostan Republic
6. Regional Pediatric Clinical Hospital
7. Tyumen State Medical University, Ministry of Healthcare of Russian Federation
8. Ryazan State Medical University, , Ministry of Healthcare of Russian Federation
9. Regional Clinical Hospital №1
10. Regional Pediatric Hospital
11. Altai State Medical University, Ministry of Healthcare of Russian Federation
Issue: Vol 19, No 4 (2020): supplement
Pages: 18-29
Section: MATERIALS OF THE 3rd WORKING MEETING OF ALLERGISTS-IMMUNOLOGISTS
Submitted: 07.01.2021
Published: 07.01.2021
URL: https://hemoncim.com/jour/article/view/443
DOI: https://doi.org/10.24287/1726-1708-2020-19-4suppl-18-29
ID: 443

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Abstract

According to the data from Russian primary immunodeficiencies (PID) registry 71% of registered patients were treated with immunoglobulins (IG). Regular immunoglobulin substitutions were reported in 90% of patients with primary antibody deficiencies (PAD), 86% - with syndromic PID and 91% of patients with combined T and B cell defects. The study was supported by Academic Council of Dmitry Rogachev National Medical Center of Pediatric Hematology, Oncology and Immunology and approved by Local Ethical Committee within the Russian PID registry. Regular IG substitution was analyzed in the representative cohort of 235 PID patients from 12 Russian regions. Of these 121 were children, 114 – adults. In 78% cases IG treatment has been started during the first 10 years of life. 80% patients were treated with highly safe products (Octagam 5% and 10%, Privigen, IG VENA, Gamunex) reaching therapeutic median pre-infusion level of serum IgG of 7 g/l. Significantly lower levels of pre-infusion serum IgG were observed in patients treated with Gabreglobin-IgG. Irregular treatment was observed in 61% of patients mainly due to the poor drug supply (lack of medication in the health care centers). Infections were reported in 90% percent of patients with irregular treatment. Unscheduled hospitalizations were two times more frequent in the group of patients with irregular IVIG treatment. Additionally, we assessed quality of life of patients with regular IVIG treatment, which significantly improved in comparison with the pretreatment period and became comparable to the group of healthy controls.

Keywords

primary immunodeficiency, registry, intravenous immunoglobulin, trough level, side effects, substitution therapy, quality of life

About the authors

A. A. Mukhina

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

Author for correspondence.
Email: ffmanya@yandex.ru
ORCID iD: 0000-0002-3305-1694

a researcher at the Department of Epidemiology and Monitoring of Primary Immunodeficiencies, Department of Immunology

1 Samory Mashela St., Moscow 117997, Russia

Russian Federation

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