Gamma interferon in patients with inborn errors of immunity

Introduction: Inborn errors of immunity due to alterations in the gamma interferon pathways cause severe and recurrent infections.
Objective: To evaluate the efficacy of gamma interferon in hyper IgE syndrome and in chronic granulomatous disease.
Presentation of the cases: Two-year-old girl, with hyper IgE syndrome of autosomal dominant inheritance, with a history of recurrent abscesses and very pruritic generalized erythroderma, skeletal
manifestations and immunological alterations determined by decreased concentrations of IgG 5.1 g/L (7.81-15.30 g/L) and IgM: 0.21 g/L (0.69-2.69g/L), with normal IgA, from: 078 g/L (0.50-2, 30 g/L ea increased IgE, 532 IU (> 200 IU). Lymphocyte subpopulations by flow cytometry revealed decreased percentages of CD3+CD4+ T lymphocytes: 21.34% (25-50%), normal CD3+CD8+ T lymphocytes: 12.60% (11-32%) and lymphocytes B CD19+: 22.75% (17-41%), as well as increased CD3-CD56+ natural killer cells and natural killer T cells: 18.19% (3-15%) and 25.12% (0. 9-20.1%), respectively.
The second case was a male patient with Chronic Granulomatous Disease, with multiple staphylococcal abscesses of the skin and liver, chronic gingivitis, mycobacterial infections, and BCG reaction. Deficiencies of phagocytic cells and T lymphocytes were found, with normal immunoglobulins. Both patients were treated with gamma interferon, reducing the frequency and severity of infections and allergic manifestations. Conclusion: Gamma interferon is effective in patients with inborn errors of immunity.

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