Abdominal involvement in pediatric heart and lung transplant recipients with posttransplant lymphoproliferative disease increases the risk of mortality

• Published in: Journal of pediatric surgery Vol. 43; no. 12; pp. 2174 - 2177
• Main Authors: Tai, Cindy C, Curtis, Jennifer L, Szmuszkovicz, Jacqueline R, Horn, Monica V, Ford, Henri R, Woo, Marlyn S, Wang, Kasper S
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.12.2008
• Subjects: Adolescent; Age Factors; Child; Child, Preschool; Disease Transmission, Infectious; Epstein-Barr Virus Infections - etiology; Epstein-Barr Virus Infections - pathology; Epstein-Barr Virus Infections - transmission; Female; Heart Transplantation - adverse effects; Heart-Lung Transplantation - adverse effects; Humans; Immunocompromised Host; Immunosuppressive Agents - adverse effects; Immunosuppressive Agents - therapeutic use; Infant; Intestinal Perforation - etiology; Intestinal Perforation - surgery; Intestines - pathology; Intestines - surgery; Intussusception - etiology; Intussusception - surgery; Lung Transplantation - adverse effects; Lymphoproliferative Disorders - drug therapy; Lymphoproliferative Disorders - etiology; Lymphoproliferative Disorders - mortality; Lymphoproliferative Disorders - pathology; Male; Pediatric heart transplantation; Pediatric lung transplantation; Pediatrics; Postoperative Complications - drug therapy; Postoperative Complications - etiology; Postoperative Complications - mortality; Postoperative Complications - pathology; Postoperative Complications - surgery; Posttransplant lymphoproliferative disease; Retrospective Studies; Surgery; Posttransplant lymphoproliferative disease; Pediatric heart transplantation; Pediatric lung transplantation
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2008.08.044

Abstract Background Posttransplant lymphoproliferative disease (PTLD) is a serious complication in transplant recipients. Abdominal PTLD has been reported, but the prognosis remains undefined. The purpose of this study was to identify the incidence, predisposing factors, and outcome of abdominal PTLD in pediatric cardiothoracic transplant patients. Methods Retrospective chart review of 134 transplant patients (50 heart, 77 lung, 7 heart/lung) at our institution (1995-2005). Results Posttransplant lymphoproliferative disease was diagnosed in 14 patients. Most were Epstein-Barr virus naive initially, but all had seroconverted when diagnosed with PTLD. Eight had abdominal involvement; 4 required surgical interventions—1 for intussusception and for bowel perforation, 2 for bowel perforation, and 1 for tumor debulking. All had lifelong follow-up, with an average follow-up of 3 years. Of 8 patients with abdominal PTLD, 4 died of complications related to PTLD, whereas 1 of 6 patients with extraabdominal PTLD died of PTLD. Conclusions Epstein-Barr virus infection after transplantation is a major risk factor for PTLD. Pediatric patients with PTLD who present with abdominal involvement are more likely to die of PTLD than those without abdominal disease. Delay in diagnosis may contribute to the high mortality. Therefore, prompt evaluation and surveillance for possible abdominal PTLD may decrease mortality associated with this devastating problem.