Department of Paediatrics, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Paediatric Rheumatology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Eur J Haematol. 2023 Sep;111(3):458-476. doi: 10.1111/ejh.14029. Epub 2023 Jun 21.
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).
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.
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%).
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.
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）的死亡率很高。白细胞介素-1 受体拮抗剂阿那白滞素现被推荐用于 HLH/MAS 的早期治疗，对于病情严重的患者建议静脉内（IV）使用。本系统评价旨在建立与继发性 HLH/MAS（sHLH/MAS）中 IV 用阿那白滞素相关的文献。
我们筛选了 Embase、PubMed 和 Medline，包括所有关于 HLH 或 MAS 中 IV 用阿那白滞素的报告。我们提取了年龄、HLH/MAS 触发因素、连续输注或推注给药以及存活率等数据。
29 份病例报告/系列共确定了 87 例患者（中位年龄 22 岁，范围为 22 个月至 84 岁），均为 sHLH。在可识别的触发因素中，43%为全身感染，33%为风湿性疾病，9%为肿瘤性疾病。儿童主要有风湿性疾病触发因素（48%），而成年人则更多地由感染引起（50%）。总体而言，风湿性疾病触发的疾病存活率更高（83.3%），特别是与肿瘤性触发因素相比（42.9%）。儿童的存活率更高，特别是 10 岁以下的儿童（83%，而成年人为 63%）。
尽管 IV 用阿那白滞素的接受者可能病情严重，但与大型历史队列相比，该队列的疾病触发因素和存活率相似，这提高了对年龄和触发因素特异性生存模式的认识。IV 用阿那白滞素具有广泛的治疗剂量范围和耐受性，无论触发因素如何，在严重的 sHLH/MAS 中都具有很大的实用性。
