芦可替尼一线治疗噬血细胞综合征研究进展
Progress of ruxolitinib as first-line treatment for hemophagocytic lymphohistiocytosis

孟广强.芦可替尼一线治疗噬血细胞综合征研究进展[J].内科急危重症杂志,2026,32(2):188-191

DOI：10.11768/nkjwzzzz20260217

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作者	单位	E-mail
孟广强		menggq2001@163.com

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中文摘要:

摘要噬血细胞综合征又称为噬血细胞性淋巴组织细胞增多症（HLH），是一种免疫介导的淋巴细胞和组织细胞过度活化导致的高炎症因子综合征。炎症因子风暴和由其导致的广泛器官组织损伤为HLH病理生理特征。JAK-STAT通路活化在HLH的炎症因子风暴的发生中起着关键作用。JAK1/JAK2抑制剂芦可替尼可以通过抑制JAK-STAT通路减轻炎症因子水平治疗HLH。芦可替尼主要应用于复发难治HLH的挽救治疗，近几年其一线治疗HLH的报道亦逐年增多。现将芦可替尼一线治疗HLH的机制、临床研究和并发症进行综述。

英文摘要:

Abstract Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a syndrome of high inflammatory factors caused by immune mediated overactivation of lymphocytes and histiocytosis. The inflammatory factor storm and its resulting extensive organ and tissue damage are the pathophysiological features of HLH. JAK STAT pathway activation plays a key role in the occurrence of inflammatory cytokine storms in HLH. The JAK1/JAK2 inhibitor ruxolitinib can treat HLH by inhibiting the JAK STAT pathway to reduce levels of inflammatory cytokines. Ruxolitinib is mainly used in the salvage treatment of relapse refractory HLH. In recent years, the studies of first line treatment of HLH by ruxolitinib have increased year by year. This article reviews the mechanism, clinical studies and complications of first line treatment of HLH with ruxolitinib.