Wolcott-Rallison syndrome - crosstalk between PERK- EIF2A and type II interferon signaling.

Immune activation is not reported in children with Wolcott-Rallison syndrome (WRS). We observed a pattern of immune activation pre-transplant in three children with WRS that was consistent with increased interferon-γ (IFN-γ) and its regulated chemokine, CXCL9 in plasma, and bone marrow that demonstrated scattered hemophagocytosis. One child developed hemophagocytic lymphohistiocytosis after liver transplantation that was fatal. Children with WRS should undergo evaluation for inflammatory biomarkers before transplant to aid in diagnosis of and prompt treatment of hyperinflammatory syndromes. Early recognition can prevent fatal outcomes.