Haploidentical hematopoietic stem cell transplantation for pediatric patients with chronic active Epstein-Barr virus infection:a retrospective analysis of a single center
作者机构：Beijing Key Laboratory of Pediatric Hematology OncologyNational Key Discipline of Pediatrics(Capital Medical University)Key Laboratory of Major Diseases in ChildrenMinistry of EducationHematology Oncology CenterDepartment of Hematology and OncologyBeijing Children's HospitalCapital Medical UniversityNational Center for Children’s HealthNanlishi Road No.56Beijing 100045China
出 版 物：《World Journal of Pediatrics》 (世界儿科杂志（英文版）)
年 卷 期：2021年第17卷第6期
页 面：626-636页
核心收录：
学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学]
基 金：National Science and Technology Key Projects, (2017ZX09304029001) Beijing Municipal Science and Technology Commission, BMSTC, (Z171100001017050) Beijing Municipal Science and Technology Commission, BMSTC
主 题：Chronic active Epstein-Barr virus infection Haploidentical hematopoietic stem cell transplantation Prognosis Graft versus host diseases Thrombotic microangiopathy
摘 要：Background This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation(haplo-HSCT)for the treatment of pediatric patients with chronic active Epstein-Barr virus infection(CAEBV).Methods Children with CAEBV who did not have matched donors and underwent haplo-HSCT in Beijing Children s Hospital,Capital Medical University,from October 2016 to June 2020 were analyzed *** relating to the clinical manifestations,engraftment,and prognosis of the children were extracted from medical *** Twenty-five patients,including 16 males and 9 females,with an onset age of 5.0±2.6 years and a transplantation age of 6.9±2.9 years,were enrolled irnhis *** mean time from diagnosis to transplantation was 3.8(2.0-40.2)*** mean observation time was 19.0±12.0 *** patients received the reduced intensity conditioning regimen,and the remaining patients all received the modified myeloablative conditioning *** the end of the follow-up,23 patients were characterized by disease-free survival(DFS),22 were characterized by event-free survival(EFS).and two *** of the patients died of thrombotic microangiopathy(TMA),and another died of graft versus host disease(GVHD);this patient discontinued the treatment for economic *** 3-year overall survival(OS)rate was estimated to be 92.0%±5.4%,and the 3-year EFS rate was estimated to be 87.4%±6.8%.All active patients survived after HSCT *** GVHD degrees 1-3 were observed in ten patients(40.0%),and degree IV was observed in six(24.0%),who were all cured except for one *** GVHD was observed in nine(36.0%),and most of these cases were *** incidence of TMA and veno-occlusive disease(VOD)was 28.0%and 4.0%.Conclusions Haploidentical hematopoietic stem cell transplantation is safe and effective in the treatment of pediatric CAEBV and can be used as an alternative therapy without matched donors or emergency tr
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