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. This evidence concerns the gene IFNA1 and Decreased total neutrophil count.