Cen Tianqi, Xuan Weixia, Huang Shaohui, Wang Ziqi, Shen Lijun, Zhang Moyuan, Fang Jinzhou, Yang Shenying, Zhang Xiaoju
Department of Respiratory and Critical Care Medicine, Xinxiang Medical University, Henan Provincial People's Hospital, Zhengzhou, China.
Department of Respiratory and Critical Care Medicine, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Zhengzhou, China.
Front Immunol. 2024 Nov 15;15:1483257. doi: 10.3389/fimmu.2024.1483257. eCollection 2024.
Hemophagocytic syndrome (HPS) is a rapidly progressive and highly fatal disease, and is even more complex when it occurs during pregnancy. Currently, the HLH-94 protocol is commonly used for treatment for HPS, with ruxolitinib being mostly used for salvage therapy. Here, we report a pregnant woman who presented with fever, thrombocytopenia, splenomegaly, and subsequently developed into severe pneumonia and multiple organ dysfunction(MODS). The patient was diagnosed as HPS based on clinical manifestations, laboratory indexes, and hemophagocytosis observed in bone marrow aspirate smear. After receiving ruxolitinib as induction therapy combined with HLH-94 protocol, the patient significantly improved and eventually cured.
噬血细胞综合征（HPS）是一种进展迅速且死亡率很高的疾病，在孕期发生时情况更为复杂。目前，HLH - 94方案常用于HPS的治疗，芦可替尼大多用于挽救治疗。在此，我们报告一名孕妇，她出现发热、血小板减少、脾肿大，随后发展为重症肺炎和多器官功能障碍（MODS）。根据临床表现、实验室指标以及骨髓穿刺涂片观察到的噬血细胞现象，该患者被诊断为HPS。在接受芦可替尼作为诱导治疗并联合HLH - 94方案后，患者病情显著改善并最终治愈。
