The perfect storm

Abstract

In this issue of Blood, Tsoukas et al report that 2 distinct defects in immune pathways, cytotoxicity, and autoinflammation can synergize to produce a spontaneous hemophagocytic lymphohistiocytosis (HLH). This observation significantly advances our pathogenic model for understanding how and why HLH presents in certain patients. This in turn opens new avenues for addressing these pathways diagnostically and therapeutically.