背景:噬血细胞性淋巴组织细胞增生症(HLH)或巨噬细胞活化综合征(MAS)具有潜在的高死亡率。Anakinra,白细胞介素-1受体拮抗剂,现在建议在HLH/MAS早期,建议在严重不适的患者中使用静脉(IV)。本系统综述建立了与继发性HLH/MAS(sHLH/MAS)中的IVanakinra相关的文献。
方法:我们筛选了Embase,PubMed,还有Medline,包括用于HLH或MAS的IVanakinra的所有报告。我们提取了年龄,HLH/MAS触发器,连续输注或推注给药,和生存。
结果:29例病例报告/系列确定了87例患者(中位年龄22岁,范围为22个月至84年),所有与sHLH。在可识别的触发器中,43%为全身感染,33%风湿病,9%肿瘤。儿童主要有风湿病触发因素(48%),而成年人更常见的是感染驱动(50%)。总的来说,风湿病触发的疾病显示出更高的生存率(83.3%),特别是与肿瘤触发因素相比(42.9%)。孩子们有更大的存活率,特别是10年以下(83%,vs.成年人,63%)。
结论:尽管接受anakinraIV的患者可能严重不适,与大型历史队列相比,该队列具有相似的疾病触发因素和生存率,并增强对年龄和特定于触发因素的生存模式的认识。IVanakinra具有广泛的治疗剂量范围和耐受性,不管触发,在严重的sHLH/MAS中表现出实质性的效用。
BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) has a potentially high mortality rate. Anakinra, an interleukin-1 receptor antagonist, is now recommended early in HLH/MAS, with intravenous (IV) use proposed in critically unwell patients. This systematic
review establishes the literature relating to IV anakinra in secondary HLH/MAS (sHLH/MAS).
METHODS: We screened Embase, PubMed, and Medline, including all reports of IV anakinra for HLH or MAS. We extracted age, HLH/MAS trigger, continuous infusion or bolus dosing, and survival.
RESULTS: Twenty-nine case reports/series identified 87 patients (median age 22 years, range 22 months to 84 years), all with sHLH. Amongst identifiable triggers, 43% were systemic infection, 33% rheumatological, 9% oncological. Children had predominantly a rheumatological trigger (48%), whilst adults were more commonly infection-driven (50%). Overall, rheumatologically triggered disease showed greater survival (83.3%), particularly compared with oncological triggers (42.9%). Children had a greater survival, particularly under 10 years (83%, vs. adults, 63%).
CONCLUSIONS: Despite IV anakinra recipients likely to be critically unwell, this cohort had similar disease triggers and survival compared to large historical cohorts, and enhances awareness of age and trigger-specific survival patterns. IV anakinra had a wide therapeutic dosing range and tolerability, regardless of trigger, demonstrating substantial utility in severe sHLH/MAS.