Diagnosis and treatment of posttransplant lymphoproliferative disorder after pediatric liver transplantation
10.3760/cma.j.issn.0254-1785.2019.07.006
- VernacularTitle:儿童肝移植术后淋巴组织增殖性疾病临床分析
- Author:
Jingyi LIU
1
;
Liying SUN
;
Zhijun ZHU
;
Lin WEI
;
Ying LIU
;
Zhigui ZENG
;
Wei QU
;
Enhui HE
;
Ruifang XU
;
Yafei HE
Author Information
1. 首都医科大学附属北京友谊医院肝脏移植中心 首都医科大学儿童肝脏移植临床诊疗与研究中心 国家消化系统疾病临床医学研究中心 100050
- Keywords:
Liver transplantation;
Lymphoproliferative disorder;
Epstein-Barr virus
- From:
Chinese Journal of Organ Transplantation
2019;40(7):404-409
- CountryChina
- Language:Chinese
-
Abstract:
Objective To retrospectively explore the clinical symptoms ,diagnosis ,treatment and prognosis of posttransplant lymphoproliferative disorder (PTLD) after pediatric liver transplantation .Methods The diagnosis and treatment of PTLD were reviewed for 3 children recipient with living donor liver transplantation .Their primary diseases were biliary atresia ,glycogen storage disease type III and ornithine-transcarbamylase deficiency . All of them received FK506 for immunosuppression therapy . They were diagnosed as PTLD at 7 ,8 ,6 months post-operation respectively .Their major clinical manifestations were non-specific ,including fever ,diarrhea and anemia .Positron emission tomography/computed tomography (PET/CT) and ultrasound revealed enlarged mesenteric lymph nodes with neck lymphoadenopathy (n=2) . Pathological examinations of resected enlarged lymph nodes indicated post-transplantation lymphoproliferative disorder .One case was diffuse large B cell lymphoma and two of them belonged to preliminary EBER + . Results After a definite diagnosis ,there was one cycle of R-CHOP regimen (rituximab ,cyclophosphamide , pirarubicin ,vincristine ,dexamethasone) or 2 cycles of rituximab along with a .reduction of anti-rejection drug and they stayed in remission .Three were followed up for 37 ,39 and 20 months respectively from May 31 , 2019 . Currently transplanted liver function was stable and EBV viral load remained negative persistently .Conclusions This case highlights the complexity of clinical presentations and co-morbidities of PTLD . Reducing immunosuppressive agents and using rituximab plus chemotherapy can achieve a satisfactory efficacy for Epstein-Barr virus-related PTLD patients after pediatric liver transplantation .
