EBV and posttransplantation lymphoproliferative disease: what to do?

This review summarizes the available evidence and outlines our approach to the prophylaxis and management of posttransplantation lymphoproliferative disorder (PTLD) in adult solid organ transplantation recipients. We attempt to reduce immunosuppression as tolerated in every patient with suspected PTLD in close cooperation with their transplantation physician. There is no evidence to guide the decision when to initiate further treatment; we usually wait no longer than 4 weeks and always initiate further therapy unless there is a complete or at least good partial remission. If clinical and histological findings indicate rapidly progressive disease, we initiate additional therapy significantly earlier. CD20-positive PTLD accounts for approximately 75% of PTLD cases. Outside of clinical trials, we currently regard sequential therapy with rituximab and CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone/prednisolone) chemotherapy as standard evidence-based treatment for CD20-positive PTLD unresponsive to immunosuppression. We also discuss our approach to the rare instance of adults with PTLD associated with primary EBV infection, localized (stage I) disease, rare PTLD subtypes, and refractory/relapsed disease based on the available retrospective data and our own experience. In addition to immunotherapy and chemotherapy, this includes local therapy approaches such as surgery and radiotherapy in stage I disease, plasmacytoma-like PTLD, and primary CNS PTLD. We also provide our view on the current indications for the use of allogeneic cytotoxic T cells, even though this treatment modality is so far unavailable in our clinical practice.