Ruxolitinib for hematopoietic cell transplantation-associated hemophagocytic lymphohistiocytosis
Ruxolitinib for hematopoietic cell transplantation-associated hemophagocytic lymphohistiocytosis
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DOI：
10.1007/s12185-020-02999-3
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发表时间：
2020-09-26
影响因子：
2.1
通讯作者：
Hasegawa, Daisuke
中科院分区：
文献类型：
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作者：
Ono, Rintaro;Ashiarai, Miho;Hasegawa, Daisuke
噬血细胞淋巴组织细胞增多症是异基因造血细胞移植后的严重并发症，可导致移植失败或多器官衰竭。在这里，我们报告两名儿童难治性血细胞转化相关的HLH治疗鲁索利替尼。在第一位患者中，鲁索利替尼缓解了发热、细胞减少和高铁蛋白血症。在另一名患者中，尽管在服用鲁索利替尼之前发展和恶化的严重肝功能衰竭是不可逆转的，但观察到发热、白细胞减少和高铁素血症迅速改善。值得注意的是，多重细胞因子图谱显示两个患者的细胞因子风暴均有改善。鲁索利替尼可能是HCT相关HLH的一种令人鼓舞的选择。
Hemophagocytic lymphohistiocytosis (HLH) is a severe complication after allogeneic hematopoietic cell transplantation (HCT) and can cause graft failure or multi-organ failure. Here, we report two children with refractory HCT-associated HLH treated with ruxolitinib. In the first patient, ruxolitinib resolved fever, cytopenia and hyperferritinemia. In another patient, although severe hepatic failure, which developed and worsened before the administration of ruxolitinib, was irreversible, rapid improvement in fever, leukopenia and hyperferritinemia was observed. Of note, multiplex cytokine profiling showed amelioration of cytokine storm in both patients. Ruxolitinib may be an encouraging option for HCT-associated HLH.