儿童造血干细胞移植后EB病毒相关淋巴组织增殖性疾病的诊疗进展

Recommendation for diagnosis and treatment of Epstein-Barr virus related post-transplant lymphoproliferative disorder following hematopoietic stem cell transplant in children

EB病毒相关淋巴组织增殖性疾病(EBV-PTLD)是儿童造血干细胞移植(HSCT)后的严重并发症之一。减低强度的预处理(RIC)、抗胸腺细胞球蛋白(ATG)的使用、移植物抗宿主病(GVHD)、巨细胞病毒感染等多种高危因素与儿童EBV-PTLD发生密切相关。由于目前移植后EBV-PTLD相对少见,且缺乏统一的治疗方案,因此儿童EBV-PTLD临床诊疗极具挑战性。文章旨在综述儿童EBV-PTLD的发病机制、危险因素、诊断标准及最新治疗进展,为进一步完善适用于儿童EBV-PTLD的诊疗规范提供建议。

The Epstein-Barr virus related post-transplant lymphoproliferative disorder(EBV-PTLD)is one of the deadliest complications after hematopoietic stem cell transplantation in children.Multiple risk factors have been associated with the onset of EBV-PTLD such as reduced intensity conditioning(RIC),use of antithymocyte globulin(ATG),graft-versus-host disease(GVHD),cytomegalovirus reactivation,etc.There is no clear consensus on the treatment of EBV-PTLD in children transplant recipients due to few clinical trials and the rarity of the disease.This article aims to explore the entity of EBV-PTLD in childhood hematopoietic stem cell transplantation recipients,expanding on pathogenesis,risk factors,clinical diagnosis and recent treatment strategy,as such to highlight the gaps in knowledge needed for building a perfect treatment paradigm suitable for all pediatric patients.