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

Abstract

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.

Keywords

• Primary immunodeficiency
• Immunoglobulin
• treatment
• side effect

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