Department of Hematology, Tongji Hospital, Tongji Medical College, Hua Zhong University of Science and Technology, Wuhan, Hubei, China; Department of Hematology, Hubei TCM hospital, Wuhan, Hubei, China.
Department of Hematology, Tongji Hospital, Tongji Medical College, Hua Zhong University of Science and Technology, Wuhan, Hubei, China.
Semin Hematol. 2020 Jan;57(1):26-30. doi: 10.1053/j.seminhematol.2018.07.006. Epub 2019 Feb 27.
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.
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会导致严重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，多达 30%的患者对一线治疗没有反应。我们报告了 3 例由淋巴瘤（2 例）和成人Still 病引起的继发性 HLH/巨噬细胞活化综合征。他们接受了低剂量芦可替尼加 HLH-94 方案治疗，治疗反应迅速，无明显不良反应。治疗后，多种疾病标志物（包括发热、纤维蛋白原、血清铁蛋白和肝功能检查）均有所改善。我们的报告表明，低剂量芦可替尼加 HLH-94 方案治疗可能是继发性 HLH 的一种潜在选择，需要进一步进行临床试验来验证这种治疗方案。
