Incidence and outcome of primary Epstein-Barr virus infection and lymphoproliferative disease in pediatric heart transplant recipients

Background: The objective of this study was to assess the relationship betw een Epstein-Barr virus (EBV) infection and posttransplantation lymphoprolif erative disease (PTLD) in pediatric heart transplant recipients. EBV is imp licated in the development of PTLD. However, the relationship between prima ry EBV infection and PTLD is not well understood. Methods: Serial EBV titers were determined prospectively in 50 children bef ore and after heart transplantation Results were correlated with the develo pment of PTLD. The clinical presentation, management, and outcome of PTLD w ere characterized. Results: Before transplantation, EBV titers were positive in 19 and negativ e in 31 patients. After transplantation, all EBV-positive patients remained positive; I developed PTLD. Among EBV-negative patients, 12 of 31 remained negative; none developed PTLD. Nineteen patients demonstrated serologic ev idence of primary EBV infection after heart transplantation; 12 developed P TLD. Mean follow-up after heart transplantation was 3.3 years (range 0.4 to 8.4 years). Mean time from heart transplantation to histologic confirmatio n of PTLD was 29 months (range 3 to 72 months). Survival with PTLD was 92%. Conclusions: Twelve of 13 pediatric heart transplant recipients who develop ed PTLD had evidence of primary EBV infection. Serial monitoring of EBV tit ers may lead to earlier identification and improved treatment of PTLD.