Epstein-Barr Virus associated Post-Transplant Lymphoproliferative Disorder

Record number: 
541
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
In adults, rates range from 1%–3% in kidney and liver transplants, 1%–6% in cardiac transplants, 2%–6% in combined heart–lung transplants, 4%–10% in lung transplants, and in up to 20% of small intestine transplants.
Time to detection: 
1 year
Alerting signals, symptoms, evidence of occurrence: 
The most common presenting features of PTLD are fever and lymphadenopathy, though extranodal involvement occurs in more than two thirds of cases and may also involve the allograft, particularly in lung and small bowel transplant recipients. The central nervous system (CNS) is frequently involved (in up to 30% of cases) and can be the only site of disease.
Demonstration of imputability or root cause: 
Post-transplant lymphoproliferative disorders (PTLD) after solid organ transplantation is usually of donor origin, and is associated with Epstein-Barr virus (EBV) that leads to uncontrolled B cell proliferation and tumour formation. The higher incidence of PTLD in paediatric transplant recipients is attributable in large part to the development of primary EBV infection after transplantation. EBV seronegative adults who acquire primary EBV infection after transplantation are also at increased risk of PTLD, but since most adults are already EBV seropositive at the time of transplantation, this is less of a problem. Tissue from the recipient should be subjected to standard histology, examined for the presence of EBV by immunostaining or in-situ hybridisation, cellular infiltrates characterised by relevant phenotypic markers and clonality estimated.
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