Abstract
PurposeWe 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).MethodsA 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.ResultsThe median age of the 35 patients was 7.0 years old (range 2.5–17.5 years). 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 months (2–50 months). The 3-year overall survival rates in patients treated with chemotherapy only (n = 6) and chemotherapy followed by HSCT (n = 25) 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 × 105 copies/ml vs. 1.84 × 106 copies/ml, Mann–Whitney U: P = 0.0004; 5.00 × 102 copies/ml vs. 3.17 × 103 copies/ml, Mann–Whitney U; P = 0.003; 2.27 × 105 copies/ml vs. 1.84 × 106 copies/ml, P = 0.0001; 5.00 × 102 copies/ml vs. 3.17 × 103 copies/ml, P = 0.003). 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 cm vs. 1.9 cm, P = 0.003; 3.8 cm vs. 0 cm, P < 0.008). 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 = 0.689; virological response: 90.9% vs. 76.9%, P = 0.337).ConclusionThe L-DEP regimen is an effective therapy in CAEBV for bridging to allo-HSCT.
Highlights
Chronic active Epstein–Barr virus infection (CAEBV) is a rare lymphoproliferative disorder (LPD) that typically presents as persistent infectious mononucleosis-like disease and/or haemophagocytic lymphohistiocytosis (HLH) [1,2,3]
Eighteen of them had higher loads in all subtypes of lymphocytes, including 10 patients mainly infected with NK cells and 8 patients mainly infected with T cells
After EBV infection, proliferating NK cells were mainly positive in 4 patients, T cells in 23 patients, and T-NK cells in 8 patients
Summary
Chronic active Epstein–Barr virus infection (CAEBV) is a rare lymphoproliferative disorder (LPD) that typically presents as persistent infectious mononucleosis-like disease and/or haemophagocytic lymphohistiocytosis (HLH) [1,2,3]. This disease occurs mainly due to inflammation accompanied by EBV infection of T or NK cells. EBV-infected T or NK cells can proliferate and infiltrate clonally into multiple organs, leading to different clinical behaviours from indolent disease to rapidly life-threatening disease. Allogeneic HSCT (allo-HSCT) is the only curative treatment for eliminating EBV-infected T or NK cells [4]. To improve the prognosis, patients should receive chemotherapy before allo-HSCT to resolve disease activity [1]
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