Hepatoportal venopathy due to disseminated Mycobacterium avium complex infection in a child with IFN-gamma receptor 2 deficiency.

The control of intracellular microorganisms such as mycobacteria is largely dependent on the adaptive immune response, specifically the interaction of T helper cells and antigen presenting cells such as macrophages. The interferon gamma (IFN-gamma) pathway activation is crucial for containment and killing of mycobacteria, as evidenced by the fact that defects in this pathway often result in profound infections with both tuberculous and non-tuberculous mycobacteria. We herein report a case of a child with autosomal recessive IFN-gamma receptor 2 (IFN-gammaR2) deficiency who developed hepatic venopathy secondary to disseminated Mycobacterium avium complex (MAC) infection.