Objective: To summarize the incidence, diagnosis and treatment experience of posttransplant lymphoproliferative diseases {PTLD) in the pediatric liver transplant recipients.
Methods: We retrospectively analyzed the clinical data of pediatric liver transplant recipients. The incidence, clinical symptoms, laboratory and imaging data of PTLD in pediatric liver transplant recipients were collected. The pathological results and treatment methods were analyzed. The prognosis was evaluated.
Results: A total of 749 pediatric liver transplantation patients were treated at Beijing Friendship Hospital from June 2013 to July 2021, and PTLD was confirmed in 45 patients (19,42.2% , male; 8,17.8%, donation after death), the incidence of PTLD was 6.0% (45/749) in children after liver transplantation. The median age of PTLD patients was 10.3 months (range, 4.6-146.7 mo). The median time for EBV DNA replication was 2.9 months (range, 0.9-35.1 mo) after the operation, and the median time of onset was 14.6 months (range, 1.2-46.5 mo) after the operation. 90.2% (41/45) of patients with PTLD had superficial lymphadenopathy. Pathological results of 93.3% (42/45) patients showed positive EBER by in situ hybridization. In 43 patients, positron emission computed tomography (PET)-CT revealed increased FDG metabolism in the associated enlarged lymph nodes. All 45 patients were treated with reduced immunosuppressive drugs, and some of the patients were treated with targeted therapy, chemotherapy, surgical resection and adoptive immunotherapy depends on its pathological types. Twelve patients were in stable condition, two patients died of progressive disease, and 31 patients achieved complete or partial remission. After treatment with reduction in immunosuppression, rejection occurred in 6 patients, and liver function improved after administration of the immunosuppressive drug.
Conclusions: Primary EBV infection and immunosuppression after liver transplantation in children may increase the risk of PTLD. The possibility of PTLD should be considered in nonspecific symptoms with increasing EBV DNA load and superficial lymphadenopathy. Monitoring EBV DNA replication load and decreasing immunosuppression are essential methods to treat PTLD in children after liver transplantation. However, after reducing the level of immunosuppression, we should pay close attention to the liver function and guard against rejection.
Key words: Pediatric Liver transplantation; Posttransplant lymphoproliferative disease; Epstein-Barr virus infection; reduction in immunosuppression