Treatment of Disseminated Mycobacterial Infection with High-Dose IFN-γ in a Patient with IL-12Rβ1 Deficiency

Abstract EN

IFN-γ has been used in the treatment of IL-12Rβ1 deficiency patients with disseminated BCG infection (BCGosis), but the optimal dose to reach efficacy is not clear.

We used IFN-γ in the treatment of a 2.7-year-old patient with IL-12Rβ1 deficiency and refractory BCG-osis.

IFNγ was started at a dose of 50 μg/m2 3 times per week.

The dose was upgraded to 100 mcg/m2 after 3 months, then to 200 mcg/m2 6 months afterwards.

Serum mycobactericidal activity and lymphocytes number and function were evaluated throughout the study.

There was no clinical response to IFN-γ with 50 or 100 μg/m2 doses.

However, there was some response to the 200 μg/m2 dose with no additional adverse effects.

The serum mycobactericidal activity was not significantly different during the whole treatment period.

Lymphocytes proliferation in response to PHA was significantly higher after 3 months of using the highest dose as compared to the lowest dose.

The tuberculin skin test reaction remained persistently negative.

We conclude that in a patient with IL-12Rβ1 deficiency, IFN-γ at a dose of 200 μg/m2, but not at lower dosages, was found to have a noticeable clinical effect with no additional adverse effects.