Yuanyuan Hou, Yiwei Yan, Xiuli Zhu, Jian Chen, Yu Zheng, Yang Li, Wenting Zhang, Xiaofei Lu, Yuqiao Diao, Lian Jiang
{"title":"Clinical characteristics and prognosis of EBV-Positive mature B-Cell lymphoma in children: a retrospective study.","authors":"Yuanyuan Hou, Yiwei Yan, Xiuli Zhu, Jian Chen, Yu Zheng, Yang Li, Wenting Zhang, Xiaofei Lu, Yuqiao Diao, Lian Jiang","doi":"10.1007/s00277-025-06506-8","DOIUrl":null,"url":null,"abstract":"<p><p>Epstein-Barr virus (EBV)-associated pediatric mature B-cell lymphoma is characterized by rapid progression, a poor prognosis, and a high relapse rate. However, research on this condition remains limited. This retrospective study analyzed its clinical features and prognostic factors in children. Kaplan‒Meier analysis was performed to assess the impact of tumor EBV-encoded RNA (EBER)impact status on survival, and LASSO Cox regression was used to identify risk factors. The predictive performance of a model including the identified risk factors was assessed using calibration curves and receiver operating characteristic (ROC) curves. Tumor EBER status was significantly positively correlated with the EBV-DNA level (P < 0.05). EBER-positive pediatric patients presented elevated IgA levels, reduced IgG/IgM levels, decreased CD4 + T-cell counts, and increased CD8 + T-cell counts (P < 0.05). Compared with EBER-negative patients, EBER-positive patients had worse 5-year cumulative progression-free survival (PFS) (73.33%) and cumulative overall survival (OS) (78.30%) (P < 0.05). LASSO Cox regression analysis identified EBER (PFS HR 3.48, 95% CI 1.16-10.47, P = 0.027; OS HR 8.10, 95% CI 1.80-36.38, P = 0.006) and LDH (PFS HR 3.05, 95% CI 1.07-8.68, P = 0.037; OS HR 7.38, 95% CI 1.42-38.47, P = 0.018) as key prognostic markers. The model predicted 3-/5-year survival with high accuracy, with a C-index of 0.699 (95% CI: 0.558-0.841) for PFS and AUC (95% CI) values of 0.730 (0.575-0.886) and 0.691 (0.515-0.868). For OS, the C-index was 0.810 (95% CI: 0.669-0.952), and the AUC (95% CI) values were 0.805 (0.605-1.005) and 0.817 (0.618-1.015). Pediatric patients with EBV-associated mature B-cell lymphoma present distinct clinical and prognostic features that significantly influence the tumor immune microenvironment. The risk prognostic model based on tumor EBER status and LDH level reliably predicts outcomes in these patients.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0000,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Hematology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00277-025-06506-8","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Epstein-Barr virus (EBV)-associated pediatric mature B-cell lymphoma is characterized by rapid progression, a poor prognosis, and a high relapse rate. However, research on this condition remains limited. This retrospective study analyzed its clinical features and prognostic factors in children. Kaplan‒Meier analysis was performed to assess the impact of tumor EBV-encoded RNA (EBER)impact status on survival, and LASSO Cox regression was used to identify risk factors. The predictive performance of a model including the identified risk factors was assessed using calibration curves and receiver operating characteristic (ROC) curves. Tumor EBER status was significantly positively correlated with the EBV-DNA level (P < 0.05). EBER-positive pediatric patients presented elevated IgA levels, reduced IgG/IgM levels, decreased CD4 + T-cell counts, and increased CD8 + T-cell counts (P < 0.05). Compared with EBER-negative patients, EBER-positive patients had worse 5-year cumulative progression-free survival (PFS) (73.33%) and cumulative overall survival (OS) (78.30%) (P < 0.05). LASSO Cox regression analysis identified EBER (PFS HR 3.48, 95% CI 1.16-10.47, P = 0.027; OS HR 8.10, 95% CI 1.80-36.38, P = 0.006) and LDH (PFS HR 3.05, 95% CI 1.07-8.68, P = 0.037; OS HR 7.38, 95% CI 1.42-38.47, P = 0.018) as key prognostic markers. The model predicted 3-/5-year survival with high accuracy, with a C-index of 0.699 (95% CI: 0.558-0.841) for PFS and AUC (95% CI) values of 0.730 (0.575-0.886) and 0.691 (0.515-0.868). For OS, the C-index was 0.810 (95% CI: 0.669-0.952), and the AUC (95% CI) values were 0.805 (0.605-1.005) and 0.817 (0.618-1.015). Pediatric patients with EBV-associated mature B-cell lymphoma present distinct clinical and prognostic features that significantly influence the tumor immune microenvironment. The risk prognostic model based on tumor EBER status and LDH level reliably predicts outcomes in these patients.
期刊介绍：
Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
