L‐DEP regimen salvage therapy for paediatric patients with refractory Epstein‐Barr virus‐associated haemophagocytic lymphohistiocytosis. (11th June 2020)

• Record Type: Journal Article
• Authors: Zhao, Yunze; Li, Zhigang; Zhang, Li; Lian, Hongyun; Ma, Honghao; Wang, Dong; Zhao, Xiaoxi; Zhang, Qing; Wang, Tianyou; Zhang, Rui
• Is Part Of: British journal of haematology. Volume 191:Number 3(2020)
• Page Start: 453
• Page End: 459
• Publication Date: 2020-06-11
• Subjects: Epstein‐Barr virus -- haemophagocytic lymphohistiocytosis -- L‐DEP -- PEG‐asparaginase; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.16861
• Languages: English
• ISSNs: 0007-1048

Abstract

Summary: 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.