Hemophagocytic Lymphohistiocytosis (HLH) Due to Fulminant Salmonella Sepsis in the Setting of IL12Rβ1 (Interleukin 12 Receptor Beta 1) Deficiency.

Interleukin 12 receptor beta 1 (IL12Rβ1) deficiency is the most common cause of Mendelian susceptibility to mycobacterial disease (MSMD). MSMD usually predisposes the affected individuals to infections with weakly virulent mycobacteria such as Bacille Calmette-Guérin (BCG), environmental mycobacteria, non-typhoidal Salmonella, and certain other intracellular pathogens. MSMD usually presents with disseminated BCG infection after exposure to the BCG vaccine.