L-DEP regimen salvage therapy for paediatric patients with refractory Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis.

Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is one of the most common subtypes of secondary HLH. However, more than 30% of patients do not respond to traditional treatment. Here, we investigated the efficacy and safety of the L-DEP regimen as a salvage therapy for paediatric refractory EBV-HLH. We retrospectively analysed 26 paediatric patients with refractory EBV-HLH who received the L-DEP regimen at Beijing Children's Hospital from 1 January 2016 to 31 March 2019. Five of the patients achieved complete response (CR) and 11 achieved partial response (PR), indicating an overall response rate of 61·5% (CR + PR). Ten of the 16 patients who achieved CR or PR received allogenic haematopoietic stem cell transplantation (allo-HSCT), and seven were still alive at the last follow-up on 30 April 2020. Two of the 10 patients who did not respond were alive after allo-HSCT. Major side effects included increased amylase, bone marrow suppression and coagulation disorders, though these could be controlled by supportive therapy in most cases. Thus, we conclude that the L-DEP regimen is an effective and relatively safe salvage therapy for children with refractory EBV-HLH. This regimen also provides a bridge to allo-HSCT.

Zhao Y ，Li Z ，Zhang L ，Lian H ，Ma H ，Wang D ，Zhao X ，Zhang Q ，Wang T ，Zhang R ... -  《-》

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》

[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 ... -  《-》

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 ... -  《-》

[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 ... -  《-》