Low dose ruxolitinib plus HLH-94 protocol: A potential choice for secondary HLH
Low dose ruxolitinib plus HLH-94 protocol: A potential choice for secondary HLH
复制标题
低剂量鲁索替尼联合 HLH-94 方案：继发性 HLH 的潜在选择
DOI：
10.1053/j.seminhematol.2018.07.006
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发表时间：
2020-01-01
影响因子：
3.6
通讯作者：
Huang, Wei
中科院分区：
文献类型：
--
作者：
Wang, Han;Gu, Jia;Huang, Wei
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫介导的综合征，导致细胞因子风暴。不受控制的细胞因子风暴导致显著的组织损伤和潜在的危及生命的多器官衰竭。HLH的常规一线治疗包括HLH-94方案或HLH-2004方案。然而，高达30%的患者对一线治疗无反应。本文报告3例继发性HLH/巨噬细胞活化综合征，其中2例由淋巴瘤和成人斯蒂尔病引起。患者接受小剂量鲁索替尼联合HLH-94方案治疗，治疗反应迅速，无明显不良反应。治疗后，在几个疾病标志物中观察到改善，包括发热，纤维蛋白原，血清铁蛋白和肝功能检查。我们的报告表明，低剂量鲁索替尼联合HLH-94方案治疗可能是继发性HLH的潜在选择，临床试验值得进一步研究。(C)2019作者爱思唯尔公司出版
Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated syndrome resulting in cytokine storm. The uncontrolled cytokine storm leads to significant tissue damage and potentially life-threatening multiorgan failure. Conventional first-line treatment for HLH included HLH-94 protocol or HLH-2004 protocol. However, up to 30% of patients do not respond to first-line therapy. We reported 3 cases of secondary HLH/macrophage activation syndrome which were caused by lymphoma (2 cases) and adult-onset still's disease. They received low dose ruxolitinib plus HLH-94 protocol, and had rapid response to treatment without obvious adverse effects. Following the treatment, there was improvement seen in several disease markers, including fever, fibrinogen, serum ferritin, and liver function tests. Our report indicated that treatment with low dose ruxolitinib plus HLH-94 protocol might be a potential choice for secondary HLH, and clinical trials warrants to be further investigated in this treatment regimen. (C) 2019 The Authors. Published by Elsevier Inc.