Hemophagocytic lymphohistiocytosis:pathogenesis and diagnosis
Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening hyperinflammatory syn-drome characterized by the overactivation of cytotoxic T cells,natural killer cells,and macrophages arising in cy-tokine storms,a range of clinical symptoms,organ damage,and even death.HLH can be divided into primary and secondary,with the former having susceptibility genes(lymphocyte cytotoxicity defects,abnormalities of in-flammasome activation,etc.)and the latter being triggered by a variety of factors(infections,malignancy,iatro-genic factors,etc.).Prompt diagnosis and treatment are essential to improve survival.The HLH-2004 criteria and the Hscore are two widely used diagnostic tools in clinical practice.In this review,we discussed the etiology,laboratory tests,and diagnosis of HLH.
NETL
NSTL
万方数据
