成年人嗜血细胞性淋巴组织细胞增多症研究进展

Advances in adult hemophagocytic lymphohistiocytosis

嗜血细胞性淋巴组织细胞增多症（HLH）是一种以免疫功能极度活化导致病理性炎症反应的临床综合征。目前广泛使用的诊断标准是来源于儿童患者数据的 HLH-2004诊断标准。成年人 HLH 和儿童 HLH 相比，在病因、临床表现、实验室指标上存在不同，有学者提出了针对成年人 HLH 独特的诊断评分系统。 HLH 在成年人中表现多样化，需要根据诱发因素、疾病严重程度、遗传背景等制定个性化的治疗方案。

Hemophagocytic lymphohistiocytosis （HLH） is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation. The HLH-2004 diagnostic criteria is used widely based on the data from pediatric patients. However, there are differences between children and adults in the etiology, clinical and laboratory characteristics for HLH. Given these differences, diagnostic scoring systems unique to adult HLH have been proposed. HLH in adults is a much more heterogeneous syndrome requiring a more individualized approach depending on the underlying trigger, disease severity, as well as genetic background.