Combined Use of Emapalumab With Ruxolitinib and Dexamethasone as an Effective Treatment for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Complicated With Multiorgan Damage and Severe Infection

Juan Liang; Xiaojun Xu; Zhenjie Chen; Jia Yu; Yongmin Tang

doi:10.1097/MPH.0000000000002856

Combined Use of Emapalumab With Ruxolitinib and Dexamethasone as an Effective Treatment for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Complicated With Multiorgan Damage and Severe Infection

J Pediatr Hematol Oncol. 2024 Apr 26. doi: 10.1097/MPH.0000000000002856. Online ahead of print.

Authors

Juan Liang^{1

2}, Xiaojun Xu^{1

2}, Zhenjie Chen³, Jia Yu³, Yongmin Tang^{1

2}

Affiliations

¹ Division of hematology-oncology.
² The Pediatric Leukemia Diagnostic and Therapeutic Technology Research Center of Zhejiang Province, Hangzhou, China.
³ Department of Pediatric Intensive Care Unit, School of Medicine, Children's Hospital, Zhejiang University.

PMID: 38691058
DOI: 10.1097/MPH.0000000000002856

Abstract

Anti-interferon-γ monoclonal antibody emapalumab and JAK1/2 inhibitors ruxolitinib have been widely reported for the treatment of hemophagocytic lymphohistiocytosis (HLH) recently. These targeted drugs have fewer side effects and may provide new options for patients with HLH who are refractory to previous treatment or intolerant to chemotherapy. Herein, we reported a case of Epstein-Barr virus-related HLH, which did not respond well to HLH-94 plus ruxolitinib and developed severe fungal infection. The disease was successfully controlled after a combination therapy of emapalumab, ruxolitinib, and dexamethasone.