Zhao Lin, Yang Hui, Qu Wei-Ying, Lu Ying-Jia, Feng Zhou
Department of Haematology, Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Department of Clinical Laboratory, Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Front Oncol. 2023 Mar 2;13:1054175. doi: 10.3389/fonc.2023.1054175. eCollection 2023.
Haemophagocytic lymphohistiocytosis (HLH) is a cytokine-driven inflammatory syndrome caused by uncontrolled hypersecretion of inflammatory cytokines. Conventional first-line treatment for HLH included HLH-94 and HLH-2004 regimens. However, quite a few patients do not respond to treatment or cannot tolerate intensive chemotherapy. We reported two cases of HLH, one caused by natural killer (NK)/T-cell lymphoma and another associated with missense variants in the gene. They both received the ruxolitinib plus dexamethasone protocol and had a rapid response to treatment without obvious adverse effects. Our report indicates that treatment with ruxolitinib plus dexamethasone might be a potential option for HLH, and clinical trials warrant further investigation. In addition, the detection of HLH-related genes is necessary for the identification of late-onset familial HLH in certain settings.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由炎症细胞因子不受控制的过度分泌引起的细胞因子驱动的炎症综合征。HLH的传统一线治疗包括HLH - 94和HLH - 2004方案。然而，相当多的患者对治疗无反应或无法耐受强化化疗。我们报告了2例HLH，1例由自然杀伤（NK）/T细胞淋巴瘤引起，另1例与该基因的错义变异有关。他们均接受了鲁索替尼加地塞米松方案治疗，且对治疗反应迅速，无明显不良反应。我们的报告表明，鲁索替尼加地塞米松治疗可能是HLH的一种潜在选择，临床试验值得进一步研究。此外，在某些情况下，检测HLH相关基因对于识别迟发性家族性HLH是必要的。
