Outcome of treatment of Epstein–Barr virus‐related post‐transplant lymphoproliferative disorder in hematopoietic stem cell recipients: a comprehensive review of reported cases

Abstract: Post‐transplant lymphoproliferative disorder (PTLD) caused by Epstein–Barr virus (EBV) is an important complication in high‐risk allogeneic hematopoietic stem cell transplant (HSCT) recipients. Before the current methods of anti‐EBV therapy were introduced, the mortality from PTLD after HSCT was >80%. With current approaches the mortality from EBV‐PTLD can be significantly reduced. The published literature and meeting abstracts were reviewed to assess the impact of different management strategies against EBV‐PTLD. This analysis of reported outcomes indicates that preemptive use of rituximab and EBV‐cytotoxic T lymphocytes (CTL) significantly reduced the risk of death due to EBV‐PTLD in HSCT recipients with survival rates of 89.7% and 94.1%, respectively. Therapy of established PTLD also reduced the risk of fatal outcome. However, the overall success rates were lower than after preemptive therapy, reaching 63% and 88.2% of total EBV‐DNA clearance with rituximab and CTL therapy, respectively. A reduction of immunosuppression and/or donor lymphocyte infusion might also reduce the risk of death due to EBV‐PTLD. Although it is difficult to estimate these effects more precisely because of the frequent use of combination therapies, the responses to these modalities can be estimated to be 56.6% and 41.0%, respectively. Finally, chemotherapy seems not to contribute to improved survival of patients with PTLD after HSCT and antiviral agents are not active against PTLD.