Nasopharyngeal and CNS-PTLD after heart transplantation in a 3 year old boy: Successful remission after haploident EBNA1 specific activated T-Cell transfers

Post transplantation lymphoproliferative disease (PTLD) is a rare and often fatal lymphoma. It occurs especially in children after solid organ transplantation. We report about a 3-year-old boy who underwent heart transplantation (HTX) at the age of 2 ½ years due to severe coronary artery stenoses after Kawasaki’s disease. After successful HTX he contracted primary EBV infection with chronification and CNS affliction. Despite treatment with rituximab, ganciclovir and lowering of immunospuppression, EBV infection triggered nasopharyngeal and CNS PTLD, visible on MRI and PET-CT. Clinically, fever, inspiratory stridor, mucositis, stupor and ataxia were noted. EBV specific T-cells or antibodies could not be detected. Upon rapid clinical deterioration, father and grandmother were identified as haploident T-cell donors. Transfer of EBNA1 activated T-cells was performed three times. EBV viral load, clinical symptoms and foci on MRI and PET-CT resolved. The boy has remained in remission for 12 months now.

We conclude that EBNA1 T-cell transfer was an effective treatment for otherwise untreatable and fatal CNS PTLD after HTX in this 3 year old boy. EBNA1 activated T-Cell transfer should be considered as a treatment option in chronic EBV infection and EBV triggered PTLD lymphoma after organ transplantation.