Background: Post-transplant lymphoproliferative disorders (PTLD) are an impor tant cause of morbidity and mortality after organ transplantation. We sought to better define the prevalence, pathology, current therapeuti...Background: Post-transplant lymphoproliferative disorders (PTLD) are an impor tant cause of morbidity and mortality after organ transplantation. We sought to better define the prevalence, pathology, current therapeutic approaches, and out comes of PTLD in a large group of children who had received heart transplants. M ethods: We assessed data on patients followed up at 19 centres in the Pediatric Heart Transplant Study (PHTS) from 1993 to 2002. Probability of freedom from PTL D was assessed along with details of presentation, pathology, treatment, and out comes. Risk factors for survival and event-free survival were investigated. Findings: Of 1184 primary transplant recipients, 56 (5%) developed PTLD. Probabili ty of freedom from PTLD was 98%at 1 year, 94%at 3 years, and 92%at 5 years. M ean time to PTLD was 23.8 months. Most common sites of disease were gastrointest inal tract (n=22, 39%) and respiratory system (n=14, 25%). Histology was polym orphic in 35 (65%) and monomorphic in 19 (35%). 47 of 48 cases were of B-cell origin, 39 of 45 (87%) were Epstein-Barr virus positive. Probability of survi val was 75%at 1 year, 68%at 3 years, and 67%at 5 years after diagnosis. Death from graft loss was as frequent as death from PTLD. Interpretation: About 5%of paediatric heart-transplant recipients develop PTLD, almost always of B-cell lineage and driven by Epstein-Barr virus. Although many achieve satisfactory ou tcomes, mortality remains substantial with death due to progressive disease and allograft loss. Advances in management should focus on strategies to protect the allograft as well as improved therapies for PTLD.展开更多
文摘Background: Post-transplant lymphoproliferative disorders (PTLD) are an impor tant cause of morbidity and mortality after organ transplantation. We sought to better define the prevalence, pathology, current therapeutic approaches, and out comes of PTLD in a large group of children who had received heart transplants. M ethods: We assessed data on patients followed up at 19 centres in the Pediatric Heart Transplant Study (PHTS) from 1993 to 2002. Probability of freedom from PTL D was assessed along with details of presentation, pathology, treatment, and out comes. Risk factors for survival and event-free survival were investigated. Findings: Of 1184 primary transplant recipients, 56 (5%) developed PTLD. Probabili ty of freedom from PTLD was 98%at 1 year, 94%at 3 years, and 92%at 5 years. M ean time to PTLD was 23.8 months. Most common sites of disease were gastrointest inal tract (n=22, 39%) and respiratory system (n=14, 25%). Histology was polym orphic in 35 (65%) and monomorphic in 19 (35%). 47 of 48 cases were of B-cell origin, 39 of 45 (87%) were Epstein-Barr virus positive. Probability of survi val was 75%at 1 year, 68%at 3 years, and 67%at 5 years after diagnosis. Death from graft loss was as frequent as death from PTLD. Interpretation: About 5%of paediatric heart-transplant recipients develop PTLD, almost always of B-cell lineage and driven by Epstein-Barr virus. Although many achieve satisfactory ou tcomes, mortality remains substantial with death due to progressive disease and allograft loss. Advances in management should focus on strategies to protect the allograft as well as improved therapies for PTLD.
