Korean J Pediatr Gastroenterol Nutr.  2009 Sep;12(2):199-206.

Posttransplantation Lymphoproliferative Disorder after Liver Transplantation in Pediatric Patients: Report from a Single-center Over 21 Years

• Lee JH
• Ko JS
• Seo JK
• Yi NJ
• Suh KS
• Lee KU
• Kang GH

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.

Keyword

Posttransplantation lymphoproliferative disorder (PTLD); Pediatric liver transplantation

MeSH Terms

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
Antibodies, Monoclonal, Murine-Derived
Ganciclovir
Immunosuppressive Agents