Interleukin 1 Receptor Antagonist Anakinra, Intravenous Immunoglobulin, and Corticosteroids in the Management of Critically Ill Adult Patients With Hemophagocytic Lymphohistiocytosis
Interleukin 1 Receptor Antagonist Anakinra, Intravenous Immunoglobulin, and Corticosteroids in the Management of Critically Ill Adult Patients With Hemophagocytic Lymphohistiocytosis
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DOI：
10.1177/0885066617711386
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发表时间：
2019-09-01
影响因子：
3.1
通讯作者：
Robak, Oliver
中科院分区：
文献类型：
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作者：
Wohlfarth, Philipp;Agis, Hermine;Robak, Oliver
背景资料：噬血细胞性淋巴组织细胞增生症（HLH）导致多器官功能障碍，经常导致重症监护室（ICU）转诊和/或死亡。我们报告了一系列采用非依托泊苷为基础的方案治疗的重症成人患者，包括白细胞介素1拮抗剂阿那白滞素、静脉注射免疫球蛋白（IVIG）和/或皮质类固醇（CS）治疗HLH。研究方法：回顾性分析了2014年3月至2016年3月期间在一家大型三级护理大学医院（奥地利维也纳医科大学）接受阿那白滞素+/- IVIG +/- CS治疗HLH的8例成人（≥ 18岁）ICU患者。结果如下：8例患者（中位年龄：38年;范围：20-58岁; 4名男性和4名女性）接受阿那白滞素联合IVIG（n = 7）和/或高剂量CS（n = 5）治疗疑似反应性HLH（中位H评分：214;范围：171-288）。7例（88%）患者需要血管加压药和有创机械通气，6例（75%）患者需要肾脏替代治疗（HLH诊断时序贯器官衰竭评估[SOFA]评分中位数：9.5;范围：6-14）。6例患者在治疗开始后1周和2周SOFA评分显著下降（P = 0.03），其余2例患者发生早期死亡。5例患者存活至ICU出院，其中4例可进一步出院（住院存活率：50%）。未观察到明显的给药相关毒性。结论：阿那白滞素联合IVIG和/或CS导致8例重症成人HLH患者的住院生存率为50%，尽管存在很大程度的器官功能障碍，需要积极的ICU治疗。需要对重症成人HLH的非依托泊苷治疗策略进行进一步研究。
Background: Hemophagocytic lymphohistiocytosis (HLH) causes multiple organ dysfunction frequently leading to intensive care unit (ICU) referral and/or death. We report on a series of critically ill adult patients treated with a non-etoposide-based regimen including interleukin 1 antagonist anakinra, intravenous immunoglobulin (IVIG), and/or corticosteroids (CS) for HLH. Methods: Eight adult (>= 18 years) ICU patients having received treatment with anakinra +/- IVIG +/- CS for HLH between March 2014 and March 2016 at a large tertiary care university hospital (Medical University of Vienna, Vienna, Austria) were retrospectively analyzed. Results: Eight patients (median age: 38 years; range: 20-58 years; 4 males and 4 females) received anakinra together with IVIG (n = 7) and/or high-dose CS (n = 5) for suspected reactive HLH (median H-score: 214; range: 171-288). Seven (88%) patients required vasopressors and invasive mechanical ventilation and 6 (75%) patients required renal replacement therapy (median Sequential Organ Failure Assessment [SOFA] score at HLH diagnosis: 9.5; range: 6-14). Six patients showed a significant decline in the SOFA score at 1 and 2 weeks following treatment initiation (P = .03), and the remainder 2 patients experienced early death. Five patients survived to ICU discharge, 4 of them could further be discharged from hospital (hospital survival rate: 50%). No overt treatment-related toxicity was noted. Conclusion: Anakinra in combination with IVIG and/or CS resulted in a hospital survival rate of 50% in 8 critically ill adult patients with HLH despite a vast degree of organ dysfunction and the need for aggressive ICU treatment. Further research on non-etoposide-based treatment strategies for HLH in critically ill adults is warranted.