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学科主题: 临床医学
题名:
Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis
作者: Wang, Yini1; Huang, Wenqiu1; Hu, Liangding2; Cen, Xinan3; Li, Lihong4; Wang, Jijun5; Shen, Jianliang6; Wei, Na1; Wang, Zhao1
刊名: BLOOD
发表日期: 2015-11-05
DOI: 10.1182/blood-2015-05-644914
卷: 126, 期:19, 页:2186-2192
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Hematology
研究领域[WOS]: Hematology
关键词[WOS]: MACROPHAGE ACTIVATION ; IMMUNOCHEMOTHERAPY ; TRANSPLANTATION ; PATHOGENESIS
英文摘要:

Hemophagocytic lymphohistiocytosis (HLH) is a refractory immune disorder with a significant risk of death. Although standard therapy has dramatically improved survival in HLH patients, approximately 30%, especially adults, show no response to current treatment strategies. This prospective study aimed to investigate the efficacy of liposomal doxorubicin treatment combined with etoposide and methylprednisolone (doxorubicin-etoposide-methylprednisolone; DEP) as a salvage therapy for adult refractory HLH. Adult patients who did not achieve at least partial response 2 weeks after initial standard HLH therapy were enrolled in this study between June 2013 and June 2014. Response to salvage therapy was assessed at 2 and 4 weeks after initiation of DEP therapy and patients were followed until death or until November 2014. Sixty-three refractory HLH patients were enrolled, including 29 cases of lymphoma-associated HLH, 22 cases of Epstein-Barr virus-associated HLH, and 4 cases of familial HLH. There were 8 cases with unknown underlying diseases. Seventeen cases (27.0%) achieved complete response and 31 cases (49.2%) achieved partial response. The overall response was 76.2% (48/63). Patients who showed no response to DEP died within 4 weeks after salvage therapy. Twenty-nine of the 48 patients who achieved partial or complete response survived to subsequent chemotherapy, allogenic hematopoietic stem cell transplantation, or splenectomy. Our study suggests that DEP regimen is an effective salvage regimen for adult refractory HLH, which can prolong patient survival as we continue to understand the responsible mechanisms and bridge the gap between HLH and its underlying diseases. This study was registered in the Chinese Clinical Trials Registry Platform (http://www.chictr.org.cn/) as ChiCTR-IPC-14005514.

语种: 英语
所属项目编号: Z151100004015172 ; 81401627 ; 2014-4-2025
项目资助者: Beijing Science and Technology Plan ; National Natural Science Fund, China ; Medical Development Research Foundation of the Capital, China
WOS记录号: WOS:000366390900008
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/59755
Appears in Collections:北京大学第一临床医学院_血液内科_期刊论文

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作者单位: 1.Navy Gen Hosp, Dept Hematol, Beijing, Peoples R China
2.Capital Med Univ, Beijing Friendship Hosp, Dept Hematol, Beijing 100050, Peoples R China
3.Acad Mil Med Sci, Affiliated Hosp, Dept Transplantat, Beijing, Peoples R China
4.Peking Univ, Hosp 1, Dept Hematol, Beijing 100871, Peoples R China
5.Capital Med Univ, Beijing Chao Yang Hosp, Dept Hematol, Beijing, Peoples R China
6.Peking Univ, Hosp 3, Dept Hematol, Beijing 100871, Peoples R China

Recommended Citation:
Wang, Yini,Huang, Wenqiu,Hu, Liangding,et al. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis[J]. BLOOD,2015,126(19):2186-2192.
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