Posttransplantation lymphoproliferative disorders in pediatric thoracic organ recipients.
OBJECTIVE
To determine the frequency, predisposing factors, clinical presentation, and outcome of posttransplantation lymphoproliferative disorders (PTLDs) in pediatric thoracic organ transplant recipients.


METHODS
Retrospective review of the medical records of all 120 children who survived longer than 1 month after thoracic organ transplantation at our center.


RESULTS
PTLD was diagnosed in 14 patients (11.7%), including 7.7% of heart and 19.5% of heart-lung/lung recipients. Presentation of PTLD was variable, ranging from asymptomatic lung nodules on chest radiograph to diffuse multiorgan failure. Treatment with a reduction of immunosuppression and antiviral therapy resulted in resolution of PTLD in eight patients. Eight patients died. PTLD contributed to death in five. No patient seropositive for Epstein-Barr virus (EBV) before transplantation had PTLD. There was a significant association between primary EBV infection after transplantation and the presence of PTLD.


CONCLUSIONS
PTLD occurs with greater frequency in pediatric thoracic organ transplant recipients than in the adult transplant population. Primary EBV infection after transplantation is the major risk factor for the development of PTLD. Patients in whom primary EBV infection develops after transplantation should be managed with a reduction in immunosuppression and with heightened surveillance for the development of PTLD.
Boyle
G J
GJ
Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh, Pennsylvania, USA.
Michaels
M G
MG
Webber
S A
SA
Knisely
A S
AS
Kurland
G
G
Cipriani
L A
LA
Griffith
B P
BP
Fricker
F J
FJ
D000293:Adolescent; D000328:Adult; D000998:Antiviral Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D016027:Heart Transplantation / Q000009:adverse effects; D016041:Heart-Lung Transplantation / Q000009:adverse effects; D006566:Herpesviridae Infections / Q000150:complications / Q000188:drug therapy; D004854:Herpesvirus 4, Human; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000009:adverse effects; D008171:Lung Diseases / Q000209:etiology; D016040:Lung Transplantation / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology; D016377:Organ Transplantation / Q000009:adverse effects*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D013903:Thoracic Surgery; D016896:Treatment Outcome; D014412:Tumor Virus Infections / Q000150:complications / Q000188:drug therapy
D000998:Antiviral Agents; D007166:Immunosuppressive Agents