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

Aim: Primary immunodeficiency (PID) is a monogenic disease characterizedby susceptibility to infections, autoimmunity, and malignancy.Immunoglobulin replacement therapy (IgRT) is an effectivetreatment option for preventing infections. In this study, we aimedto present the demographic and clinical characteristics of patientswith primary immunodeficiency who received IgRT at our center.Materials and Methods: In this cross-sectional study, the filesof patients who received IgRT treatment for primary immunodeficiencyin our Pediatric Allergy and Immunology clinic between2023 and 2025 were retrospectively reviewed.Results: The study included 40 patients who received IgRT treatmentfor primary immunodeficiency. Of these 40 patients whoreceived IgRT treatment, 12 patients had unclassified hypogammaglobulinemia,12 had primary antibody deficiency (7 commonvariable immunodeficiency, 2 SPI mutation, 1 PIK3R1 mutation, 2Bruton), 5 had syndromic combined immunodeficiency (Di georgesyndrome, Ataxia-Telangiectasia, Charge Syndrome), 5 had hypogammagobulinemiaassociated with Down syndrome, 4 hadcombined immunodeficiency, and 2 had innate immune systemdefects. 13 (32.5%) of the patients had consanguineous marriage,and 6 (15%) had a family history of primary immunodeficiency. Themost common presenting complaint (70%) was recurrent infections.Anaphylaxis developed in 1 patient and urticaria in 2 patientsafter IgRT treatment. A decrease in infection frequency was observedin all patients during follow-up.Conclusion: IgRT should be individualized for each patient in thetreatment and follow-up of primary immunodeficiency. Patientsshould be closely monitored for side effects. IgRT in patients withprimary immunodeficiency is effective in reducing infections andmorbidity when indicated and diagnosed appropriately.