Low-dose emapalumab combined with chemotherapy for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

Wenjuan Zhu; Fei Zhou; Yue Song; Shiyuan Zhou; Feng Du; Qian Zhu; Ziyi Wang; Liyun Bai; Jianhong Fu; Xiao Ma; Xiaojin Wu; Xuefeng He

doi:10.1016/j.trim.2024.102162

Low-dose emapalumab combined with chemotherapy for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

Transpl Immunol. 2024 Dec 22:102162. doi: 10.1016/j.trim.2024.102162. Online ahead of print.

Authors

Wenjuan Zhu¹, Fei Zhou¹, Yue Song¹, Shiyuan Zhou¹, Feng Du², Qian Zhu², Ziyi Wang², Liyun Bai², Jianhong Fu¹, Xiao Ma¹, Xiaojin Wu³, Xuefeng He⁴

Affiliations

¹ National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China.
² Soochow Hopes Hematonosis Hospital, Suzhou 215000, China.
³ National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China. Electronic address: wuxiaojin@suda.edu.cn.
⁴ National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China. Electronic address: hexuefeng@suda.edu.cn.

PMID: 39719162
DOI: 10.1016/j.trim.2024.102162

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder with poor clinical outcomes. Use of emapalumab, an IFN-γ inhibitor, enables primary HLH control in over 85 % of affected children. However, data on emapalumab use for Epstein-Barr virus-associated HLH (EBV-HLH) are limited. This report presents the cases of three patients with EBV-HLH, highlighting the successful integration of low-dose emapalumab in combination with chemotherapy as a novel therapeutic approach for patients diagnosed with EBV-HLH. This regimen resulted in rapid disease symptom control and hematological parameter improvement and facilitated successful stem cell transplantation. This report highlights the potential of low-dose emapalumab combined with chemotherapy as an effective bridging therapy to allogenic hematopoietic stem cell transplantation in EBV-HLH patients.

Keywords: Emapalumab; Hemophagocytic lymphohistiocytosis; Stem cell transplantation.