Diagnosis and treatment of posttransplant lymphoproliferative disorder after pediatric liver transplantation

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. Key words: Liver transplantation; Lymphoproliferative disorder; Epstein-Barr virus