Posttransplantation lymphoproliferative disorder in pediatric liver transplantation

Abstract Introduction The aim of this study was to evaluate the clinical features of risk factors for posttransplantation lymphoproliferative disorder (PTLD) in pediatric liver transplantation. Materials and methods Between June 1996 and June 2002, among 41 pediatric patients who underwent liver transplantation, 7 died in the postoperative period. Thirty-five patients, including 1 patient who died of PTLD, were reviewed. Based on the serology results, patients were divided into a high-risk group (EBV-naive recipients of EBV-positive grafts) and a low-risk group (patients other than those in the high-risk group). Results Five of 41 patients (12.2%) developed PTLD. All of them belonged to the high-risk group. The incidence of PTLD in the high-risk group was 31.3% (5 of 16). The mean duration between operation and diagnosis for PTLD was 9.8 months. Primary EBV infection developed at a median of 6 months after transplantation. Three of 5 patients developed rejection before the diagnosis of PTLD. One patient was diagnosed with laryngeal and gastrointestinal PTLD, whereas the other 4 had gastrointestinal PTLD. They experienced the following symptoms and signs: anemia (100%), hypoalbuminemia (100%), fever (80%), diarrhea (80%), gastrointestinal bleeding (80%), and anorexia (60%). Conclusion The common features of PTLD development were as follows: (1) EBV-positive donors placed into EBV-naive recipients, (2) primary EBV infection approximately 6 months after transplantation, (3) young age, 1 year old at operation, and (4) requirement for intensive posttransplantation immunosuppression.