Hemophagocytic syndrome(HPS),also known as hemophagocytic lymphohistiocytosis(HLH),is a rare and highly malignant hematologic disease with a poor prognosis.It can be divided into two categories:primary HLH and secondary HLH.HLH is characterized by a large number of abnormal immune cells that continuously activate and regulate out of control,leading to systemic inflammatory factor storm and multiple organ failure.Clinical manifestations mainly include persistent malignant hyperthermia,pancytopenia,hepatosplenomegaly,and hemophagocytosis in tissues and organs.The pathogenesis of adult HLH is complex,with rapid onset and rapid disease progression,and the fatality rate remains high.The main causes of death in HLH patients are multiple organ failure,abnormal coagulation and septic shock.Due to the specificity of HLH and the lack of effective understanding of the severity and prognosis in clinical practice,some HLH patients are misdiagnosed or overlooked,missing the best opportunity for diagnosis and treatment.Therefore,this review systematically explores and discusses the latest diagnostic and treatment progress of adult HLH,aiming to provide reference for clinical diagnosis,treatment and prognosis assessment of HLH.
关键词
噬血细胞综合征/恶性血液病/诊断与鉴别/治疗/预后
Key words
hemophagocytic syndrome/malignant hematological diseases/diagnosis and differentiation/treatment/prognosis
维普
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