Post Transplant Lymphoproliferative Disease (PTLD) in Paediatric Liver Transplantation

Abstract

Introduction: Post transplant lymphoproliferative disease (PTLD) is a complication of transplantation. It is associated with Epstein-Barr virus (EBV) and manifest as a systemic disease. The reported mortality ranges from 12-35%. The clinical data on this disease is still not well documented in paediatric liver transplantation as the number of sufferers is not large. Aim of study: To evaluate the incidence, onset time after liver transplant, clinical presentation, organ involvement, and clinical outcome of PTLD in paediatric patients in our centre. Method: This is a retrospective review of the results of 95 paediatric patients who had undergone liver transplant in our centre from Sept 1993 till Dec 2009. Medical records of the patients with PTLD were retrieved. Diagnosis of PTLD was confi rmed by histology. Results: Among the 95 paediatric liver transplant patients in our centre, 8 developed PTLD with an incidence of 8.4%, which is much higher than the incidence rate of adult liver transplant patients in our centre (0.7%). Mean onset of PTLD was 19.8 months (range 4-64 months) after the liver transplant. The clinical manifestations varied widely from fever (n=3), poor appetite (n=2), peripheral lymphadenopathy (n=2) to gastrointestinal bleeding (GI) bleeding (n=2), intestinal obstruction (n=1) and multi-organ failure (n=1). Three patients had lymphoma. EBV was identifi ed from the histological specimens in all the above patients. Organ involvement includes GI tract (n=4), liver (n=2), peripheral lymph nodes (n=4) and iris (n=1). One patient had OKT3 as one of the immunosuppresants and 2 patients had acute graft rejection which required pulse steroid. One patient died before the start of medical treatment. All the other 7 patients received Rituximab (anti CD 20), and 2 of these 7 patients received additional chemotherapy. All 7 patients are alive with a mean follow up of 36.7months (range 2-99 months). One patient had recurrence of PTLD ten months after the treatment. The mortality rate was 12.5%. Conclusion: PTLD is a life threatening disease and the incidence of paediatric PTLD is much higher than in adult population. Early diagnosis and treatment with Rituximab seems to give a relatively low mortality and promising result.