Huang Zoufang, Xie Jiangbo
Department of Hematology.
Department of Intensive Care Unit, the First Affiliated Hospital of Gannan Medical University, Jiangxi, China.
Medicine (Baltimore). 2021 Mar 19;100(11):e25188. doi: 10.1097/MD.0000000000025188.
The HLH-94 protocol is a standard induction treatment for hemophagocytic lymphohistiocytosis. However, about 30% of patients may not respond. Ruxolitinib has been clinically proven to be an effective treatment for hemophagocytic lymphohistiocytosis (HLH).
A previously healthy 14-year-old girl presented to the local hospital with a 4-day history of persistent fever and sore throat.
Clinical and laboratory tests revealed fever >38.5°C, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia, and an elevated interleukin-2 receptor level.
This patient was treated with ruxolitinib and the HLH-94 protocol.
The patient's clinical and some laboratory indices improved. Unfortunately, vital signs such as respiratory function and consciousness did not improve.
This case report highlights the effect of using ruxolitinib in conjunction with the HLH-94 protocol. However, safety evaluation of this regimen was not performed because critically ill patient died too fast.
HLH - 94方案是噬血细胞性淋巴组织细胞增生症的标准诱导治疗方案。然而，约30%的患者可能无反应。鲁索替尼已在临床上被证明是治疗噬血细胞性淋巴组织细胞增生症（HLH）的有效药物。
一名既往健康的14岁女孩因持续发热和咽痛4天就诊于当地医院。
临床及实验室检查显示体温>38.5°C、肝脾肿大、全血细胞减少、高甘油三酯血症、低纤维蛋白原血症、高铁蛋白血症以及白细胞介素 - 2受体水平升高。
该患者接受了鲁索替尼及HLH - 94方案治疗。
患者的临床症状及部分实验室指标有所改善。遗憾的是，呼吸功能和意识等生命体征未改善。
本病例报告强调了联合使用鲁索替尼和HLH - 94方案的效果。然而，由于重症患者死亡过快，未对该治疗方案进行安全性评估。
