Abstract

Post-transplantation lymphoproliferative disorder (PTLD) is a complication of organ transplantation and a life-threatening condition. Children who underwent organ transplantation are at risk of developing lymphoproliferative disorders and, among them, non-Hodgkin lymphoma (NHL) is the most serious. The objective of this study was to describe the clinical course of NHL after liver and kidney transplantation. Retrospective analysis of medical records of children who underwent liver/kidney transplantation and developed NHL. Nine children were identified, all girls, 6 after liver and 3 after kidney transplantations. Age at transplantation ranged from 1 year to 13 years (median: 4 years), while age at lymphoma diagnosis from 4 to 17 years (median: 12 years). Time from transplantation to lymphoma diagnosis ranged from 7 months to 12 years (median: 9 years). All but 1 patient developed mature B-cell lymphoma, 4 children - diffuse large B-cell lymphoma (DLBCL), 2 children - Burkitt's lymphoma, 1 child - mature B-cell leukemia, 1 child - Burkitt-like lymphoma, while 1 patient was diagnosed with T-cell lymphoblastic lymphoma. High levels of Epstein-Barr virus (EBV) DNA were found in blood of 3 patients, and EBV in tissue samples was detected in 4 patients. Six patients presented with stage III and 2 with stage IV disease. Two patients had graft involvement. Three children received chemotherapy according to R-CHOP, 3 LMB protocol (2 with addition of rituximab), while 1 received CHOP and 5 courses of COP. T-cell lymphoma patient was treated with Euro-LB protocol. Six out of 8 treated patients are alive with a median follow-up of 6 years. Two children died from disease progression during treatment and 1 from cerebral herniation before starting therapy. All patients experienced at least 1 toxic episode of grade 3 and 4 according to Common Toxicity Criteria Adverse Event (CTCAE). Complications of chemotherapy were manageable and there were no transplanted organ failures. Our study provides further data on the treatment and outcome of monomorphic PTLD and indicates that it is feasible to treat solid organ recipients with multiagent chemotherapy.

Highlights

  • Post-transplantation lymphoproliferative disorders (PTLD) are one of the most severe complications of organ transplantation in children and adults

  • All but 1 patient developed mature B-cell lymphoma, 4 children – diffuse large B-cell lymphoma (DLBCL), 2 children – Burkitt’s lymphoma, 1 child – mature B-cell leukemia, 1 child – Burkitt-like lymphoma, while 1 patient was diagnosed with T-cell lymphoblastic lymphoma

  • High levels of Epstein–Barr virus (EBV) DNA were found in blood of 3 patients, and EBV in tissue samples was detected in 4 patients

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Summary

Introduction

Post-transplantation lymphoproliferative disorders (PTLD) are one of the most severe complications of organ transplantation in children and adults. Post-transplantation lymphoproliferative disorders is a life-threatening condition and the cause of mortality and morbidity in this group of patients. Children who underwent solid organ transplantation are at significant risk of developing lymphoproliferative disorders and, among them, NHL is the most serious.[2]. Treatment of such patients is challenging with regard to the choice of chemotherapy protocol and management of chemotherapy-related complications in an already vulnerable population. Post-transplantation lymphoproliferative disorder (PTLD) is a complication of organ transplantation and a life-threatening condition. Children who underwent organ transplantation are at risk of developing lymphoproliferative disorders and, among them, non-Hodgkin lymphoma (NHL) is the most serious

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