Sepsis and hemophagocytic lymphohistiocytosis-similarities and differences
袁义 1刘铜林 1林楠
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作者信息
1. 华中科技大学同济医学院附属同济医院儿童重症医学科，武汉 430030
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摘要
脓毒症为感染引发机体反应失调、全身炎症反应，导致器官功能障碍。噬血细胞综合征为各种病因作用下机体免疫调节异常引发过度炎症反应、炎症风暴、器官功能障碍。二者相似的是均为失调的过度炎症反应，不同的是免疫细胞及细胞因子的活化谱存在差异。前者病因为感染，后者病因包括基因异常、感染、风湿免疫病、恶性肿瘤等。前者为全身炎症反应，后者为极强的高炎症状态。前者抗感染治疗是关键，后者除病因治疗外，免疫抑制治疗非常重要。除休克外，中枢神经系统受累是重要死因，二者均应加强神经功能监护。 Sepsis is dysregulated host response caused by infection leading to systemic inflammation and organ dysfunction.Hemophagocytic lymphohistiocytosis（HLH）could be caused by multiple factors，leading to abnormal immune regulation and resulting in inflammatory storm and organ dysfunction.Their pathogenesis is similar that dysregulated strong inflammatory response developing in the body，but different in activation profiles of immunologic cells and cytokines.Sepsis is caused by infection，while the etiology of HLH includes various conditions such as genetic defects，infection，rheumatic disease，malignancies，etc.There are differences in the degree of inflammation between the two diseases，with sepsis being systemic inflammation and HLH being extremely strong hyperinflammation.Anti-infection is the key to the treatment of sepsis，while immunosupressive measures are essential for HLH except for etiological treatment.Besides shock，central nervous system involvement is significant cause of death，and neuromonitoring should be applied aggressively.
Abstract
Sepsis is dysregulated host response caused by infection leading to systemic inflammation and organ dysfunction.Hemophagocytic lymphohistiocytosis（HLH）could be caused by multiple factors，leading to abnormal immune regulation and resulting in inflammatory storm and organ dysfunction.Their pathogenesis is similar that dysregulated strong inflammatory response developing in the body，but different in activation profiles of immunologic cells and cytokines.Sepsis is caused by infection，while the etiology of HLH includes various conditions such as genetic defects，infection，rheumatic disease，malignancies，etc.There are differences in the degree of inflammation between the two diseases，with sepsis being systemic inflammation and HLH being extremely strong hyperinflammation.Anti-infection is the key to the treatment of sepsis，while immunosupressive measures are essential for HLH except for etiological treatment.Besides shock，central nervous system involvement is significant cause of death，and neuromonitoring should be applied aggressively.
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