Outcome of L-DEP regimen for treatment of pediatric chronic active Epstein–Barr virus infection

Abstract

Purpose We intended to investigate the clinical features of paediatric patients with chronic active Epstein–Barr virus infection (CAEBV) and to examine the effectiveness of the L-DEP regimen before haematopoietic stem cell transplantation (HSCT). Methods A retrospective analysis was performed on 35 patients with CAEBV at Beijing Children’s Hospital from January 2016 to January 2020. The efficacy and adverse events of the L-DEP regimen were evaluated. Results The median age of the 35 patients was 7.0\xa0years old (range 2.5–17.5\xa0years). Twenty-eight patients achieved a clinical response (80.0%, 22 in clinical CR, 6 in clinical PR) after L-DEP. In terms of virological response, 7 patients (20%) were assessed as having virological CR, and 23 patients (65.7%) had virological PR. Finally, 29 patients underwent allo-HSCT. The median survival time was 18\xa0months (2–50\xa0months). The 3-year overall survival rates in patients treated with chemotherapy only (n\u2009=\u20096) and chemotherapy followed by HSCT (n\u2009=\u200925) were 33.3% and 75.4%, respectively. After L-DEP 1st treatment and L-DEP 2nd treatment, the EBV-DNA loads in blood and plasma were significantly reduced compared with those before chemotherapy (median: 4.29\u2009×\u200910 5 copies/ml vs. 1.84\u2009×\u200910 6 copies/ml, Mann–Whitney U: P \u2009=\u20090.0004; 5.00\u2009×\u200910 2 copies/ml vs. 3.17\u2009×\u200910 3 copies/ml, Mann–Whitney U; P \u2009=\u20090.003; 2.27\u2009×\u200910 5 copies/ml vs. 1.84\u2009×\u200910 6 copies/ml, P \u2009=\u20090.0001; 5.00\u2009×\u200910 2 copies/ml vs. 3.17\u2009×\u200910 3 copies/ml, P \u2009=\u20090.003). Compared with the liver and spleen size before chemotherapy, the size of the liver and spleen shrank significantly after L-DEP 2nd (median 3.8\xa0cm vs. 1.9\xa0cm, P \u2009=\u20090.003; 3.8\xa0cm vs. 0\xa0cm, P \u2009<\u20090.008). In addition, after L-DEP treatment, there was no difference in the clinical or virological response rate regardless of HLH status (clinical response: 77.3% vs. 84.6%, P \u2009=\u20090.689; virological response: 90.9% vs. 76.9%, P \u2009=\u20090.337). Conclusion The L-DEP regimen is an effective therapy in CAEBV for bridging to allo-HSCT .