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Posttransplant lymphoproliferative disorder (PTLD) is a rare but potentially serious complication following transplantation with an overall incidence of PTLD of 1–5% in solid organ transplant (SOT) recipients and 1% in hematopoietic stem cell transplant (HSCT) recipients. The clinical and pathological spectrum of PTLD is broad; however, most cases of PTLD occur within the first year after transplantation and are associated with EBV. Clinical features that independently predict rates of response and survival have not been systematically studied for PTLD. Patients whose PTLD expressed CD20 or EBV have shorter intervals to PTLD onset, whereas late-onset cases of PTLD are typically EBV negative. Phenotypic characterization of PTLD reveals potential reliance on EBV or NF-kappaB signaling instead of B-cell receptor signaling, which links PTLD to other subgroups of EBV-related lymphomas, highlighting new potential treatment approaches. PTLD can be a life-threatening post-HSCT complication due to the impact of the patient’s underlying disease (malignant or nonmalignant) as well as the type and intensity of the conditioning regimen. EBV-negative PTLD is more often a delayed phenomenon post-HSCT compared to EBV-positive PTLD. Further investigations are needed to better under stand the role of EBV in the pathogenesis of different forms of PTLD in the immunosuppressed patients.

Clinical and Pathological Review of Post Transplant Lymphoproliferative Disorders