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

IF 1.6 4区 医学 Q4 IMMUNOLOGY Transplant immunology Pub Date : 2024-12-22 DOI:10.1016/j.trim.2024.102162
Wenjuan Zhu, Fei Zhou, Yue Song, Shiyuan Zhou, Feng Du, Qian Zhu, Ziyi Wang, Liyun Bai, Jianhong Fu, Xiao Ma, Xiaojin Wu, Xuefeng He
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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.

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小剂量emapalumab联合化疗治疗eb病毒相关噬血细胞淋巴组织细胞增多症的成人患者
噬血细胞性淋巴组织细胞增多症(HLH)是一种临床预后较差的严重疾病。使用emapalumab(一种IFN-γ抑制剂)可以使85%以上的受影响儿童的原发性HLH得到控制。然而,emapalumab用于eb病毒相关HLH (EBV-HLH)的数据有限。本报告介绍了3例EBV-HLH患者的病例,强调了低剂量emapalumab联合化疗作为EBV-HLH患者的新治疗方法的成功整合。该方案导致疾病症状快速控制和血液学参数改善,并促进成功的干细胞移植。该报告强调了低剂量emapalumab联合化疗作为EBV-HLH患者同种异体造血干细胞移植的有效桥接治疗的潜力。
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来源期刊
Transplant immunology
Transplant immunology 医学-免疫学
CiteScore
2.10
自引率
13.30%
发文量
198
审稿时长
48 days
期刊介绍: Transplant Immunology will publish up-to-date information on all aspects of the broad field it encompasses. The journal will be directed at (basic) scientists, tissue typers, transplant physicians and surgeons, and research and data on all immunological aspects of organ-, tissue- and (haematopoietic) stem cell transplantation are of potential interest to the readers of Transplant Immunology. Original papers, Review articles and Hypotheses will be considered for publication and submitted manuscripts will be rapidly peer-reviewed and published. They will be judged on the basis of scientific merit, originality, timeliness and quality.
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