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成人噬血细胞综合征的诊疗
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Abstract:
噬血细胞综合症(HLH)是一种以高免疫反应为特征的危及生命的疾病。临床表现为持续发热、脾大、血细胞减少、高甘油三酯血症、低纤维蛋白原血症和高铁蛋白血症。其发病机制是遗传基因突变和外在触发因素的结合。成人HLH用皮质类固醇联合或不联合依托泊苷治疗是主要的治疗方法。
Hemophagocytic syndrome (HLH) is a life-threatening disease characterized by a high immune response. The clinical manifestations include persistent fever, splenomegaly, decreased blood cells, hypertriglyceridemia, hypofibrinogenemia, and ferritinemia. The pathogenesis is a combination of genetic mutations and external triggering factors. The main treatment method for adult HLH is the combination or non combination of corticosteroids and etoposide.
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