Treatment of disseminated mycobacterial infection with high-dose IFN-γ in a patient with IL-12Rβ1 deficiency

Clin Dev Immunol. 2011;2011:691956. doi: 10.1155/2011/691956. Epub 2010 Dec 22.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • BCG Vaccine / adverse effects*
  • Child, Preschool
  • Female
  • Humans
  • Interferon-gamma / administration & dosage
  • Interferon-gamma / adverse effects
  • Interferon-gamma / therapeutic use*
  • Lymphocyte Activation
  • Mycobacterium Infections / drug therapy*
  • Mycobacterium Infections / immunology
  • Mycobacterium Infections / microbiology
  • Mycobacterium bovis / drug effects*
  • Mycobacterium bovis / immunology
  • Receptors, Interleukin-12 / deficiency*
  • Receptors, Interleukin-12 / genetics
  • Treatment Outcome

Substances

  • BCG Vaccine
  • IL12RB1 protein, human
  • Receptors, Interleukin-12
  • Interferon-gamma