Intravenous anakinra for the treatment of haemophagocytic lymphohistiocytosis/macrophage activation syndrome: A systematic review.

Abstract

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.

• Authors: James E G Charlesworth, Akhila Kavirayani
• Journal: European journal of haematology (Eur J Haematol) Vol. 111 Issue 3 Pg. 458-476 (Sep 2023) ISSN: 1600-0609 [Electronic] England
• PMID: 37344166 (Publication Type: Systematic Review, Journal Article)
• Copyright: © 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Chemical References

• Interleukin 1 Receptor Antagonist Protein

Topics

• Adult
• Child
• Humans
• Infant
• Lymphohistiocytosis, Hemophagocytic (diagnosis, drug therapy, etiology)
• Macrophage Activation Syndrome (diagnosis, drug therapy, etiology)
• Interleukin 1 Receptor Antagonist Protein (adverse effects)
• Sepsis (complications)
• Rheumatic Diseases (complications, drug therapy)