Pediatric hemophagocytic syndrome (HS) is a distinct clinical entity in which excessive uncontrolled activation and proliferation of T cells and macrophages occur and are often fatal. To date, this syndrome remains ill-recognized in children, leading to false or delayed diagnosis and suboptimal management. HS is a component of several inherited disorders in which it is present at onset or during the course of the disease.
This syndrome has been given several other denominations like hemophagocytic histiocytosis, histiocytic disorder, macrophage activation syndrome, and reactive hemophagocytic lymphohistiocytosis (HLH). It has also been associated with a variety of viral, bacterial, fungal, and parasitic infections, as well as with collagen-vascular diseases and malignancies, particularly T-cell malignancies. Etiopathogenesis In response to infection, innate and adaptive elements of the…
