Our study showed that the patient with PAMI exhibited strong inflammatory signals in neutrophils including over production of proinflammatory cytokines IL-1β and IL-18, hyperactivation of pyrin inflammasome, and excess cell death and upregulation of NF-κB and IFN signaling pathways, which resulted in severe inflammation and may contribute to develop neutropenia in patients with PAMI syndrome. Here, IFNA1 is linked to neutropenia.