Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis
1
Issued Date
2023-01-01
Resource Type
ISSN
09024441
eISSN
16000609
Scopus ID
2-s2.0-85164102187
Pubmed ID
37385631
Journal Title
European Journal of Haematology
Rights Holder(s)
SCOPUS
Bibliographic Citation
European Journal of Haematology (2023)
Suggested Citation
Lee B.J., Cao Y., Vittayawacharin P., É'Leima G., Rezk S., Reid J., Brem E.A., Ciurea S.O., Kongtim P. Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis. European Journal of Haematology (2023). doi:10.1111/ejh.14030 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/87997
Title
Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis
Author's Affiliation
Other Contributor(s)
Abstract
Objective: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening, hyperinflammatory syndrome usually treated with high-dose steroids (HDS), often complemented with adjunct therapies, such as etoposide (HLH-94 protocol). Anakinra has been reported to effectively treat HLH; however, has not been comparatively examined with etoposide-based therapies. We sought to evaluate the effectiveness and durability of these treatment approaches. Methods: We performed a retrospective analysis of all adult patients diagnosed with secondary HLH between January 2011 and November 2022 who received anakinra and HDS, the HLH-94 protocol, HDS alone, or supportive care. Results: Thirty adult patients with secondary HLH were included. Cumulative incidence (CI) of response at 30 days was 83.3%, 60%, and 36.4% for patients treated with anakinra, the HLH-94 protocol, and HDS alone, respectively. CI of relapse at 1 year was 50%, 33.3%, and 0% with the HLH-94 protocol, HDS, and anakinra and HDS, respectively. Overall survival at 1 year was higher with anakinra and HDS compared to the HLH-94 protocol, yet was not statistically significant (77.8% vs. 33.3%; hazard ratio: 0.29; p =.25). Conclusion: Treatment with anakinra and HDS in adults with secondary HLH was associated with higher response rates with longer survival compared with alternative therapies and should be further investigated in this setting.