Posttransplantation Lymphoproliferative Disorder after Liver Transplantation in Pediatric Patients: Report from a Single-center Over 21 Years.
- Author:
Jung Hwa LEE
1
;
Jae Sung KO
;
Jeong Kee SEO
;
Nam Joon YI
;
Kyung Suk SUH
;
Kuhn Uk LEE
;
Gyeong Hoon KANG
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. jkseo@snu.ac.kr
- Publication Type:Original Article
- Keywords:
Posttransplantation lymphoproliferative disorder (PTLD);
Pediatric liver transplantation
- MeSH:
Antibodies, Monoclonal, Murine-Derived;
Biopsy;
Burkitt Lymphoma;
Child;
Diarrhea;
Fever;
Ganciclovir;
Herpesvirus 4, Human;
Humans;
Immunosuppressive Agents;
Liver;
Liver Transplantation;
Lymphoproliferative Disorders;
Medical Records;
Precursor Cell Lymphoblastic Leukemia-Lymphoma;
Prevalence;
Retrospective Studies;
Risk Factors;
Sepsis;
Transplants;
Viral Load;
Rituximab
- From:Korean Journal of Pediatric Gastroenterology and Nutrition
2009;12(2):199-206
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: To analyze the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after liver transplantation in children. METHODS: From January 1988 to June 2009, we retrospectively reviewed the medical records of 8 PTLD cases among 148 pediatric patients underwent liver transplantation. The age at transplantation, time of presentation after transplantation, clinical manifestations, histologic diagnosis, results of EBV (Epstein-Barr virus) assessments, managements and outcomes of PTLD were investigated. RESULTS: The prevalence of PTLD in liver transplant pediatric recipients was 5.4% (8 of 148). The mean age of patients was 25.4+/-21.3 months (range 10 to 67 months). Seven of 8 patients (87.5%) underwent liver transplantation before 1 year of age. The common clinical presentations were persistent fever (8 of 8, 100%) and bloody diarrhea (6 of 8, 75%). PTLD was diagnosed with gastrointestinal endoscopic biopsies in five patients and surgical biopsies in three. Histologic findings showed early lesion in three patients, polymorphic in two, and monomorphic in three. Burkitt lymphoma and lymphoblastic lymphoma were found in two of 3 monomorphic patients. Seven of 8 patients were found with EBV-positive. Eight patients were treated with dose reduction of immunosuppressants and infusion of ganciclovir. Rituximab was added to four patients. PTLD were successfully managed in all patients except one who died of sepsis during chemotherapy. CONCLUSION: Major risk factor of PTLD was to undergo liver transplantation before 1 year of age. Continuous monitoring for EBV viral load and gastrointestinal endoscopic biopsy may be useful to early detection of PTLD.
