Clinical analysis of posttransplant lymphoproliferative disorder in pediatric liver transplant recipients: a report of 11 cases
10.3760/cma.j.cn421203-20211109-00274
- VernacularTitle:儿童肝移植术后淋巴组织增殖性疾病11例临床分析
- Author:
Yuchuan LI
1
;
Conghuan SHEN
;
Jiayan FENG
;
Jianshe WANG
;
Xinbao XIE
Author Information
1. 复旦大学附属儿科医院肝病中心，上海 201102
- Keywords:
Child;
Liver transplantation;
Lymphoproliferative disorders;
Epstein-Barr virus
- From:
Chinese Journal of Organ Transplantation
2022;43(5):292-297
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinicopathological characteristics, treatments and outcomes of posttransplant lymphoproliferative disorder(PTLD)in pediatric liver transplant recipients.Methods:From October 2016 to October 2021, retrospective data analysis was performed for 11 pediatric liver transplant recipients with PTLD. There were 5 males and 6 females with a diagnostic age of 1-8 years. Living donor liver transplantation(LDLT, n=9)and deceased donor liver transplantation(DDLT, n=2)were performed. All recipients received tacrolimus plus methylprednisolone. The major clinical manifestations included lymphadenopathy, splenomegaly, anemia, fever and digestive system symptoms(diarrhea, abdominal pain, ascites, hematochezia & intussusception, etc.). Laboratory tests hinted at hypoproteinemia, elevated transaminases and serum positivity of EBV-DNA. Positron emission tomography and computed tomography(PET-CT)revealed PTLD( n=9). Ten children were diagnosed by pathology, including lymphoid hyperplasia( n=3), plasmacytic hyperplasia PTLD( n=1), polymorphic PTLD( n=2), diffuse large B-cell lymphoma( n=2), infectious mononucleosis PTLD( n=1)and Burkitt lymphoma( n=1). Results:After a definite diagnosis of PTLD, tacrolimus was tapered or discontinued. And rituximab was prescribed. Two patients received chemotherapy(R-COP & R-CHOP)while 2 cases of local masses were operated. Up until February 2022, 10 cases survived and their conditions improved. One patient died of infection.Conclusions:PTLD is one of the most serious and fatal complications after liver transplantation in children. Clinical manifestations are diverse and an early diagnosis is difficult. The changes of EBV-DNA load should be closely monitored after liver transplantation. Imaging and pathological examinations may aid in an early diagnosis of PTLD. A treatment regimen based on immunosuppression reduction and rituximab improves the prognosis of PTLD in pediatric liver transplant recipients.
