L-DEP regimen is effective as an initial therapy for adult EBV-HLH
L-DEP regimen is effective as an initial therapy for adult EBV-HLH
复制标题
L-DEP 方案作为成人 EBV-HLH 的初始治疗是有效的
DOI：
10.1007/s00277-022-04946-0
复制
发表时间：
2022-09-12
影响因子：
3.5
通讯作者：
Wang，Zhao
中科院分区：
文献类型：
--
作者：
Chen，Leilei;Wang，Jingshi;Wang，Zhao
我们进行了一项单中心前瞻性试验，研究聚乙二醇天冬酰胺酶联合脂质体阿霉素、依托泊苷和甲基强的松龙(L-DEP)作为初始治疗EB病毒驱动的噬血细胞淋巴组织细胞增生症(EBV-HLH)的疗效。在2019年9月至2021年9月期间，所有患者在确诊为EBV-HLH后都没有接受过任何化疗。在开始L-DEP初级治疗后2周和4周进行疗效评估。47名符合条件的EBV-HLH患者入选。L-DEP方案化疗后2周总有效率为80.9%(38/47，临床完全缓解12例，临床部分缓解26例)，4周总有效率为75.6%(34/45，临床完全缓解21例，临床部分缓解13例)。L方案治疗2周和4周后，全血和血浆EB病毒载量均显著下降(P<； 0.001)。治疗后2周和4周，铁蛋白、可溶性CD25、甘油三酯、超声脾长度和厚度均显著下降(P<； 0.001)。因此，L-DEP方案是治疗EB病毒-HLH的一种有效的初始治疗方案。然而，L-DEP方案的远期预后较差，一旦完全缓解，应尽快接受allo-HSCT。
We performed a single-center, prospective trial to investigate the efficacy of PEG- asparaginase combined with liposomal doxorubicin, etoposide, and methylprednisolone (L-DEP) as an initial therapy for Epstein–Barr virus driven hemophagocytic lymphohistiocytosis (EBV-HLH). None of the patients received any chemotherapy after the diagnosis of EBV-HLH between September 2019 and September 2021. The efficacy was evaluated 2 weeks and 4 weeks after initiating L-DEP primary therapy. Forty-seven eligible patients with EBV-HLH were enrolled. The overall response rate (ORR) was 80.9% (38/47, 12 in clinical CR, 26 in clinical PR) at 2 weeks after the L-DEP regimen; at 4 weeks, the ORR was 75.6% (34/45, 21 in clinical CR, 13 in clinical PR). EBV-DNA loads in blood and plasma were significantly decreased 2 and 4 weeks after the L-DEP regimen (P< 0.001). Ferritin, soluble CD25 (sCD25), triglycerides (TGs), and ultrasonic spleen longitude, and thickness were all decreased significantly 2 and 4 weeks after the L-DEP regimen (P< 0.001). Thus, the L-DEP regimen is an effective initial therapy for EBV-HLH. However, the L-DEP regimen was poor in terms of long-term prognosis and that allo-HSCT should be received as soon as possible once a complete response is achieved.