Autoimmune-associated hemophagocytic syndrome/macrophage activation syndrome

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH) encompasses an infrequent group of non-malignant, yet potentially life-threatening disorders caused by massive cytokine release from activated lymphocytes and macrophages (Filipovich, 2009; Henter et al., 1998, 2007; Janka et al., 1998; Janka, 2009). This multisystem inflammatory syndrome is associated with a range of genetic and acquired factors. Hectic and persistent fever, cytopenias, hepatitis, jaundice, edema, splenomegaly, neurological symptoms and hemophagocytosis in bone marrow (BM), liver or lymph nodes are common clinicopathological features of HLH.NonePublishe