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Korean J Pediatr Gastroenterol Nutr. 2003 Mar;6(1):39-46. Korean.
Published online Mar 31, 2003.  https://doi.org/10.5223/kjpgn.2003.6.1.39
Copyright © 2003 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
Posttransplant Lymphoproliferative Disorder in Pediatric Liver Transplantation: Samsung Medical Center Experience
Yon Ho Choe, Suk Koo Lee,* Jeong Meen Seo,* Jae Won Joh,* Sung Joo Kim,* Kwang Woong Lee,* Je Hoon Park,* Young Hye Ko, and Ki Young Kwon
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
*Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Abstract

PURPOSE

In a retrospective study for the pediatric patients who underwent liver transplantation in the past 6 years at Samsung Medical Center, the clinical features of 5 patients with posttransplant lymphoproliferative disorder (PTLD) were analyzed.

METHODS

Between June 1996 and June 2002, 41 pediatric patients underwent liver transplantation. Seven of them died in the postoperative period. Thirty-five including one patient who died of PTLD were finally reviewed. Patients were divided into two groups: high risk group, EBV naive recipients of EBV-positive grafts; low risk group, the patients other than those in high risk group. The authors reviewed age at operation, immunosuppressive agent, postoperative duration until diagnosis, postoperative duration until EBV seroconversion, presence of treatment against rejection, and presenting symptoms of PTLD.

RESULTS

Five of 41 patients (12.2%) developed PTLD. All of them belonged to high risk group, and the incidence of PTLD in high risk group was 31.3% (5/16). The mean age at operation was 10.8 months old and the mean duration between operation and diagnosis for PTLD was 9.8 months. Primary EBV infection developed after a median of 6 months after transplantation. One patient was diagnosed as laryngeal and gastrointestinal PTLD and the other four, gastrointestinal PTLD. The following symptoms and signs were seen in the patients: anemia (100%), hypoalbuminemia (100%), fever (80%), diarrhea (80%), gastrointestinal bleeding (80%), and anorexia (60%).

CONCLUSION

PTLD is one of the major complications after pediatric liver transplantation, especially in the group of high-risk recipients. Anemia, hypoalbuminemia, fever, diarrhea and gastrointestinal bleeding were features that are characteristic of PTLD. The common features of PTLD development were: (i) EBV-positive donors placed into EBV naive recipients, (ii) primary EBV infection about 6 months after transplantation, (iii) young age, about 1 year old at operation, and (iv) the requirement for intensive posttransplant immunosuppression.

Keywords: Posttransplant lymphoproliferative disorder; Pediatric liver transplantation; Epstein-Barr virus infection; Immunosuppression; Hypoalbuminemia; Anemia; Gastrointestinal bleeding