He Kang, Xu Shanshan, Shen Lijing, Chen Xiaosong, Xia Qiang, Qian Yongbing
Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.
Shanghai Engineering Research Center of Transplantation and Immunology, Shanghai 200127, China.
J Clin Med. 2022 Oct 26;11(21):6308. doi: 10.3390/jcm11216308.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal hyperinflammatory disorder characterized by dysfunctional cytotoxic T and natural killer cells. Liver transplantation is a predisposing factor for HLH. High mortality rates were reported in 40 cases of HLH following liver transplantation in adults and children. Herein, we describe a case of adult HLH triggered by cytomegalovirus (CMV) infection shortly after liver transplantation. The patient was successfully treated with ruxolitinib combined with a modified HLH-2004 treatment strategy. Our case is the first to report the successful use of ruxolitinib with a modified HLH-2004 strategy to treat HLH in a solid organ transplantation recipient.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能致命的高炎症性疾病，其特征为细胞毒性T细胞和自然杀伤细胞功能失调。肝移植是HLH的一个诱发因素。据报道，成人和儿童肝移植后发生的40例HLH死亡率很高。在此，我们描述了一例肝移植后不久由巨细胞病毒（CMV）感染引发的成人HLH病例。该患者采用鲁索替尼联合改良的HLH-2004治疗策略成功治愈。我们的病例是首例报告在实体器官移植受者中成功使用鲁索替尼联合改良的HLH-2004策略治疗HLH。
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