Immunoglobulin Replacement Therapy in Patients with Primary Immundeficiency: A Single-Center Experience

Yıl 2026, Cilt: 16 Sayı: 1, 17 - 22, 19.03.2026

Betül Keser Halime Helin Demir Dilara Kurt Ayşen Çetemen Hayrunnisa Bekis Bozkurt

https://izlik.org/JA72WG65GM

Öz

Aim: Primary immunodeficiency (PID) is a monogenic disease characterized
by susceptibility to infections, autoimmunity, and malignancy.
Immunoglobulin replacement therapy (IgRT) is an effective
treatment option for preventing infections. In this study, we aimed
to present the demographic and clinical characteristics of patients
with primary immunodeficiency who received IgRT at our center.
Materials and Methods: In this cross-sectional study, the files
of patients who received IgRT treatment for primary immunodeficiency
in our Pediatric Allergy and Immunology clinic between
2023 and 2025 were retrospectively reviewed.
Results: The study included 40 patients who received IgRT treatment
for primary immunodeficiency. Of these 40 patients who
received IgRT treatment, 12 patients had unclassified hypogammaglobulinemia,
12 had primary antibody deficiency (7 common
variable immunodeficiency, 2 SPI mutation, 1 PIK3R1 mutation, 2
Bruton), 5 had syndromic combined immunodeficiency (Di george
syndrome, Ataxia-Telangiectasia, Charge Syndrome), 5 had hypogammagobulinemia
associated with Down syndrome, 4 had
combined immunodeficiency, and 2 had innate immune system
defects. 13 (32.5%) of the patients had consanguineous marriage,
and 6 (15%) had a family history of primary immunodeficiency. The
most common presenting complaint (70%) was recurrent infections.
Anaphylaxis developed in 1 patient and urticaria in 2 patients
after IgRT treatment. A decrease in infection frequency was observed
in all patients during follow-up.
Conclusion: IgRT should be individualized for each patient in the
treatment and follow-up of primary immunodeficiency. Patients
should be closely monitored for side effects. IgRT in patients with
primary immunodeficiency is effective in reducing infections and
morbidity when indicated and diagnosed appropriately.

Anahtar Kelimeler

Primary immunodeficiency , Immunoglobulin , treatment , side effect

Kaynakça

• 1. Yu JE. New primary immunodeficiencies 2023 update. Curr Opin Pediatr. 2024;36(1):112–123. https://doi.org/10.1097/MOP.0000000000001315
• 2. Reda SM, El-Ghoneimy DH, Afifi HM. Clinical predictors of primary immunodeficiency diseases in children. Allergy Asthma Immunol Res. 2013;5(2):88–95. https://doi.org/10.4168/aair.2013.5.2.88
• 3. Goussault H, Salvator H, Catherinot E, Chabi ML, Tcherakian C, Chabrol A, et al. Primary immunodeficiency-related bronchiectasis in adults: comparison with bronchiectasis of other etiologies in a French reference center. Respir Res. 2019;20(1):1–10. https://doi.org/10.1186/s12931-019-1242-4
• 4. Picard C, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency 2015. J Clin Immunol. 2015;35(8):696–726. https://doi.org/10.1007/s10875-015-0201-1
• 5. Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, et al. Human inborn errors of immunity: 2022 update on the classification from the international union of immunological societies expert committee. J Clin Immunol. 2022;42(7):1473–1507. https://doi.org/10.1007/s10875-022-01289-3
• 6. Costagliola G, Consolini R. Primary immunodeficiencies: pathogenetic advances, diagnostic and management challenges. J Clin Med. 2023;12(14):4651. https://doi.org/10.3390/jcm12144651
• 7. Compagno N, Malipiero G, Cinetto F, Agostini C. Immunoglobulin replacement therapy in secondary hypogammaglobulinemia. Front Immunol. 2014;5:626. https://doi.org/10.3389/fimmu.2014.00626
• 8. Bruton OC. Agammaglobulinemıa. Pediatrics. 1952;9(6):722–728. https://doi.org/10.1542/peds.9.6.722
• 9. Sil A, Basu S, Joshi V, Pilania RK, Siniah S, Suri D, et al. Immunoglobulin replacement therapies in inborn errors of immunity: a review. Front Pediatr. 2024;12:1368755. https://doi.org/10.3389/fped.2024.1368755
• 10. Krivan G, Jolles S, Granados EL, Paolantonacci P, Ouaja R, Cissé OA, et al. New insights into the use of immunoglobulins for the management of patients with immune deficiency (PID). Am J Clin Exp Immunol. 2017;6(5):76–83.
• 11. Skoda-Smith S, Torgerson TR, Ochs HD. Subcutaneous immunoglobulin replacement therapy in the treatment of patients with primary immunodeficiency disease. Ther Clin Risk Manag. 2010;6(1):1–10. https://doi.org/10.2147/TCRM.S4353
• 12. Epland K, Suez D, Paris K. A clinician’s guide for administration of high-concentration and facilitated subcutaneous immunoglobulin replacement therapy in patients with primary immunodeficiency diseases. Allergy Asthma Clin Immunol. 2022;18(1):1–11. https://doi.org/10.1186/s13223-022-00726-7
• 13. Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, et al. The European society for immunodeficiencies (ESID) registry working definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract. 2019;7(6):1763–1770. https://doi.org/10.1016/j.jaip.2019.02.004
• 14. Albin S, Cunningham-Rundles C. An update on the use of immunoglobulin for the treatment of immunodeficiency disorders. Immunotherapy. 2014;6(10):1113–26. https://doi.org/10.2217/imt.14.67
• 15. Eades-Perner AM, Gathmann B, Knerr V, Guzman D, Veit D, Kindle G, et al. The European internet-based patient and research database for primary immunodeficiencies: Results 2004-06. Clin Exp Immunol. 2007;147(2):306–12. https://doi.org/10.1111/j.1365-2249.2006.03292.x
• 16. Ibis IBP, Erdur B, Erdem SB, Karaman S, Gulez N, Genel F. Adverse reactions and influencing factors in children with primary immunodeficiencies receiving intravenous immunoglobulin replacement. Allergol Immunopathol (Madr). 2020;48(6):738–44. https://doi.org/10.1016/j.aller.2020.05.008
• 17. Bonilla FA, Khan DA, Ballas ZK, Chinen J, Frank MM, Hsu JT, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2014;136(5):1186–205. https://doi.org/10.1016/j.jaci.2015.04.049
• 18. King JR, Hammarström L. Newborn Screening for Primary Immunodeficiency Diseases: History, Current and Future Practice. J Clin Immunol. 2018;38(1):56–66. https://doi.org/10.1007/s10875-017-0455-x
• 19. Al-Mousa H, Al-Saud B. Primary immunodeficiency diseases in highly consanguineous populations from Middle East and North Africa: Epidemiology, diagnosis, and care. Front Immunol. 2017;8:678. https://doi.org/10.3389/fimmu.2017.00678
• 20. Nain E, Kıykım A, Kasap NA, Barış S, Özen A, Aydıner EK. Immediate adverse reactions to intravenous immunoglobulin in primary immune deficiencies: a single center experience. Turk J Pediatr. 2020;62(3):379–386. https://doi.org/10.24953/turkjped.2020.03.004
• 21. Melo KM de, Alves LM, Valente CFC, Tavares FS. One-year intravenous immunoglobulin replacement therapy: efficacy in reducing hospital admissions in pediatric patients with inborn errors of immunity. J Pediatr (Rio J). 2022;98(2):190–195. https://doi.org/10.1016/j.jped.2021.05.011
• 22. Güngör A, Yaralı N. The use of intravenous immunoglobulin therapy in a tertiary pediatric hospital. Saudi Med J. 2020;41(2):163–167. https://doi.org/10.15537/smj.2020.2.24877
• 23. Bıçakcıoğlu I, Topal E. Two years follow up of the patients who recieved IVIG treatment due to primary immune deficiency. Abant Med J. 2019;8(1):1–4. https://doi.org/10.5505/abantmedj.2019.32704
• 24. Gathmann B, Grimbacher B, Beauté J, Dudoit Y, Mahlaoui N, Fischer A, et al. The European internet-based patient and research database for primary immunodeficiencies: results 2006-2008. Clin Exp Neuroimmunol. 2009;157:3–11. https://doi.org/10.1111/j.1365-2249.2009.03954.x
• 25. Kilic SS, Ozel M, Hafizoglu D, Karaca NE, Aksu G, Kutukculer N. The prevalances and patient characteristics of primary immunodeficiency diseases in Turkey two centers study. J Clin Immunol. 2013;33(1):74–83. https://doi.org/10.1007/s10875-012-9763-3
• 26. Berger M, Cunningham-Rundles C, Bonilla FA, Melamed I, Bichler J, Zenker O, et al. Carimune NF liquid is a safe and effective immunoglobulin replacement therapy in patients with primary immunodeficiency diseases. J Clin Immunol. 2007;27(5):503–509. https://doi.org/10.1007/s10875-007-9096-9
• 27. Baris S, Abolhassani H, Massaad MJ, Al-Nesf M, Chavoshzadeh Z, Keles S, et al. The Middle East and North Africa diagnosis and management guidelines for inborn errors of immunity. J Allergy Clin Immunol Pract. 2023;11(1):158–180. https://doi.org/10.1016/j.jaip.2022.10.003
• 28. Sefer AP, Kaya MS. Effectiveness, safety, and treatment satisfaction of 20% subcutaneous immunoglobulin replacement therapy in pediatric patients with primary and secondary immunodeficiencies. Turk Arch Pediatr. 2025;60(2):217–225. https://doi.org/10.5152/TurkArchPediatr.2025.24305
• 29. Suez D, Kriván G, Jolles S, Stein M, Gupta S, Paris K, et al. Safety and tolerability of subcutaneous immunoglobulin 20% in primary immunodeficiency diseases from two continents. Immunotherapy. 2019;11(12):1057–1065. https://doi.org/10.2217/imt-2019-0057