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
PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttra...PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttransplant lymphoproliferative disorder in lung transplant recipients have not been fully characterized. METHODS: Case presentation and 16 previously reported cases of posttransplant lymphoproliferative disorder with gastrointestinal involvement are reviewed. RESULTS: Patient ages ranged from 25 to 65 (median, 52) years. Median time fromlung transplantation to onset of posttransplant lymphoproliferative disorder was 36 (range, 1-109) months; 35 percent of cases (6/17) occurred within 18 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology before transplantation. In five patients (29 percent), the posttransplant lymphoproliferative disorder also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of posttransplant lymphoproliferative disorder was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathologic changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of 17 cases (88 percent) and surgical resection in 10 (59 percent). One patient was untreated. Seven of 16 patients (44 percent) responded to treatment and 9 patients died. Median time from onset of posttransplant lymphoproliferative disorder to death was 70 (range,10-85) days. CONCLUSIONS: Posttransplant lymphoproliferative disorder with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50 percent.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.
文摘PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttransplant lymphoproliferative disorder in lung transplant recipients have not been fully characterized. METHODS: Case presentation and 16 previously reported cases of posttransplant lymphoproliferative disorder with gastrointestinal involvement are reviewed. RESULTS: Patient ages ranged from 25 to 65 (median, 52) years. Median time fromlung transplantation to onset of posttransplant lymphoproliferative disorder was 36 (range, 1-109) months; 35 percent of cases (6/17) occurred within 18 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology before transplantation. In five patients (29 percent), the posttransplant lymphoproliferative disorder also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of posttransplant lymphoproliferative disorder was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathologic changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of 17 cases (88 percent) and surgical resection in 10 (59 percent). One patient was untreated. Seven of 16 patients (44 percent) responded to treatment and 9 patients died. Median time from onset of posttransplant lymphoproliferative disorder to death was 70 (range,10-85) days. CONCLUSIONS: Posttransplant lymphoproliferative disorder with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50 percent.
