Chen Leilei, Wang Jingshi, Wang Zhao
Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing (100050), China.
Ann Hematol. 2022 Nov;101(11):2461-2470. doi: 10.1007/s00277-022-04946-0. Epub 2022 Sep 12.
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.
我们开展了一项单中心前瞻性试验，以研究聚乙二醇化天冬酰胺酶联合脂质体阿霉素、依托泊苷和甲泼尼龙（L-DEP）作为初治方案治疗爱泼斯坦-巴尔病毒驱动的噬血细胞性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，确诊为EBV-HLH的患者均未接受任何化疗。在启动L-DEP初治方案后2周和4周评估疗效。47例符合条件的EBV-HLH患者入组。L-DEP方案治疗2周时的总缓解率（ORR）为80.9%（38/47，12例临床完全缓解，26例临床部分缓解）；4周时，ORR为75.6%（34/45，21例临床完全缓解，13例临床部分缓解）。L-DEP方案治疗2周和4周后，血液和血浆中的EBV-DNA载量显著下降（P＜0.001）。L-DEP方案治疗2周和4周后，铁蛋白、可溶性CD25（sCD25）、甘油三酯（TGs）以及脾脏超声长度和厚度均显著下降（P＜0.001）。因此，L-DEP方案是治疗EBV-HLH的有效初治方案。然而，L-DEP方案的长期预后较差，一旦获得完全缓解应尽快接受异基因造血干细胞移植（allo-HSCT）。
