In addition to genetic defects associated with FHL, there are also immunodeficiency syndromes associated with HLH that impair the secretion of lytic granule contents. These include Griscelli syndrome type 2, Hermansky Pudlak type II, Chediak Higashi and X-linked lymphoproliferative disease Type 1. Griscelli syndrome type 2 and The orange triangles indicate the first and last day that daily anakinra was given. In Figure 2a and 2b the red line denotes the threshold below which diagnostic criteria is met for HLH while in Figure 2c this line denotes the threshold above which HLH criteria is met.