XLP2 is associated with a variety of other disease manifestations including atypical/mild HLH-like episodes, inflammatory bowel disease, recurrent infections, hypogammaglobulinemia, uveitis, fistulating skin disease, granulomatous hepatitis, granulomatous, and lymphocytic interstitial lung disease (14, 15, 49–52). Here, XIAP is linked to hemophagocytic syndrome.