Abdominal involvement in pediatric heart and lung transplant recipients with posttransplant lymphoproliferative disease increases the risk of mortality.
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.
Tai
Cindy C
CC
Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA. ctai@chla.usc.edu
Curtis
Jennifer L
JL
Szmuszkovicz
Jacqueline R
JR
Horn
Monica V
MV
Ford
Henri R
HR
Woo
Marlyn S
MS
Wang
Kasper S
KS
D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D018562:Disease Transmission, Infectious; D020031:Epstein-Barr Virus Infections / Q000209:etiology / Q000473:pathology* / Q000635:transmission; D005260:Female; D016027:Heart Transplantation / Q000009:adverse effects; D016041:Heart-Lung Transplantation / Q000009:adverse effects; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D007223:Infant; D007416:Intestinal Perforation / Q000209:etiology / Q000601:surgery; D007422:Intestines / Q000473:pathology* / Q000601:surgery; D007443:Intussusception / Q000209:etiology / Q000601:surgery; D016040:Lung Transplantation / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000209:etiology / Q000401:mortality / Q000473:pathology*; D008297:Male; D011183:Postoperative Complications / Q000188:drug therapy / Q000209:etiology / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies
D007166:Immunosuppressive Agents