L-DEP regimen is effective as an initial therapy for adult EBV-HLH.

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.

Chen L ，Wang J ，Wang Z 《-》

PEG-aspargase and DEP regimen combination therapy for refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Wang J ，Wang Y ，Wu L ，Zhang J ，Lai W ，Wang Z ... -  《Journal of Hematology & Oncology》

[Ruxolitinib combined with liposomal doxorubicin, etoposide, methylprednisolone+/-PEG-asparaginase in treatment of relapsed/refractory pediatric hemophagocytic lymphohistiocytosis].

Wei A ，Ma HH ，Zhang LP ，Lian HY ，Du JY ，Wang D ，Cui L ，Ou WX ，Zhao YZ ，Zhao XX ，Zhang L ，Li ZG ，Wang TY ，Zhang R ... -  《-》

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents-a life-threatening disease: analysis of 133 cases from a single center.

Lai W ，Wang Y ，Wang J ，Wu L ，Jin Z ，Wang Z ... -  《-》

[L-DEP regimen salvage therapy for refractory primary hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus infection in 4 children].

Zhao YZ ，Ma HH ，Lian HY ，Wang D ，Wang TY ，Zhang R ... -  《-》