PEG-aspargase and DEP regimen combination therapy for refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
PEG-aspargase and DEP regimen combination therapy for refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
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用于难治性爱泼斯坦 - 巴尔病毒相关的胞菌细胞淋巴淋巴结症的PEG-ASPARGASE和DEP方案组合疗法。
DOI：
10.1186/s13045-016-0317-7
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发表时间：
2016-09-09
影响因子：
28.5
通讯作者：
Wang Z
中科院分区：
文献类型：
--
作者：
Wang J;Wang Y;Wu L;Zhang J;Lai W;Wang Z
EB病毒相关性噬血细胞性淋巴组织细胞增生症（EBV-HLH）是由感染引发的继发性HLH的最常见亚型。先前的研究表明，约30%或更多的EBV-HLH患者对标准治疗无反应。本研究探讨了改良DEP方案联合PEG-精氨酸酶（L-DEP）作为难治性EBV-HLH挽救治疗的疗效和安全性。本研究从2014年10月至2015年10月，28例难治性EBV-HLH患者在首都医科大学附属北京友谊医院接受L-DEP方案治疗。在治疗后2周和4周评价治疗效果和不良事件。接受L-DEP方案治疗前和治疗后2周的EBV-DNA浓度中位数分别为9.6 × 105（1.5 × 104 − 1 × 109）拷贝/mL和2.2 × 105（3.8 × 102 − 1.2 × 107）拷贝/mL;治疗后的数值显著低于治疗前（P = 0.048）。28例研究患者中有9例达到完全缓解（CR），15例达到部分缓解（PR），总缓解率为85.7%（CR+PR）。4名未达到反应的患者在接受L-DEP后4周内死亡。24例获得部分或完全缓解的患者中有13例接受了随后的异基因造血干细胞移植（allo-HSCT）。这13例患者中有10例存活至2016年3月1日。L-DEP方案的主要不良反应是血清淀粉酶浓度升高、肝功能异常和凝血功能障碍。这项研究表明，L-DEP是一个安全和有效的补救治疗前allo-HSCT难治性EBV-HLH，并增加了此类患者接受allo-HSCT的可能性。目前正在进行一项旨在验证L-DEP方案治疗难治性EBV-HLH的前瞻性多中心大规模临床试验（ClinicalTrails.gov标识符：NCT 02631109）。
Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is the most frequent subtype of secondary HLH triggered by infections. Previous studies have shown that ~30 % or more of patients with EBV-HLH do not respond to standard therapy. This study investigated the efficacy and safety profile of a modified DEP regimen in combination with PEG-aspargase (L-DEP) as a salvage therapy for refractory EBV-HLH. In this study from October 2014 to October 2015, 28 patients with refractory EBV-HLH received a L-DEP regimen at the Beijing Friendship Hospital, Capital Medical University. Treatment efficacy and adverse events were evaluated at 2 and 4 weeks after L-DEP treatment. Median EBV-DNA concentrations before and 2 weeks after receiving the L-DEP regimen were 9.6 × 105 (1.5 × 104 − 1 × 109) copies/mL and 2.2 × 105 (3.8 × 102 − 1.2 × 107) copies/mL, respectively; the post-treatment values were significantly lower than that of the pretreatment (P = 0.048). Nine of the 28 study patients achieved complete response (CR) and 15 partial response (PR), resulting in an overall response rate of 85.7 % (CR+PR). Four patients who did not achieve response died within 4 weeks of receiving L-DEP. Thirteen of the 24 patients who achieved partial or complete response received subsequent allogenic hematopoietic stem cell transplantation (allo-HSCT). Ten of these 13 patients survived until 1 March 2016. The major adverse effects of the L-DEP regimen were high serum amylase concentrations, abnormal liver function, and coagulation disorders. This study suggests that L-DEP is a safe and effective salvage therapy prior to allo-HSCT for refractory EBV-HLH and increases the possibility of such patients receiving allo-HSCT. A prospective multicenter large-scale clinical trial that aims to validate the L-DEP regimen for refractory EBV-HLH is currently underway (ClinicalTrails.gov Identifier: NCT02631109).
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影响因子：
3.2
作者：
Marsh, Rebecca A.;Allen, Carl E.;McClain, Kenneth L.;Weinstein, Joanna L.;Kanter, Julie;Skiles, Jodi;Lee, Nadine D.;Khan, Shakila P.;Lawrence, Julia;Mo, Jun Q.;Bleesing, Jack J.;Filipovich, Alexandra H.;Jordan, Michael B.
通讯作者：
Jordan, Michael B.
DOI：
10.3760/cma.j.issn.0253-2727.2015.06.013
发表时间：
2015-06
影响因子：
--
作者：
Zeng X;Wei N;Wang Y;Wang J;Zhang J;Wu L;Huang W;Gao Z;Pei R;Chen J;Jin Z;Wang Z
通讯作者：
Wang Z
影响因子：
12.8
作者：
Henzan, T;Nagafuji, K;Harada, M
通讯作者：
Harada, M
影响因子：
45.3
作者：
Imashuku, S;Kuriyama, K;Hibi, S
通讯作者：
Hibi, S
影响因子：
3.2
作者：
Ohga, Shouichi;Kudo, Kazuko;Hara, Toshiro
通讯作者：
Hara, Toshiro