This disease is a lymphoid proliferation or a lymphoma that develops as a consequence of immunosuppression in a patient transplanted with a solid organ or bone marrow. The histology is characterized by a proliferation of polymorphic B-cells (such as immunoblasts, plasma blasts and plasma cells). Post-transplantation lymphomas can also be monomorphic and are then classified as equivalent lymphoma (Burkitt's lymphoma, plasma cell myeloma etc.) in the WHO classification.
Post-transplantation lymphoproliferative disease (polymorphic) is often monoclonal and Epstein-Barr virus-mediated. Extra nodal manifestations occur frequently.
EBV-positive lesions occur months to years after the transplantation. Histological examinations show many variants.
The condition is fatal in about 60% of patients.
EBV-positive lesions occurring early may spontaneously disappear by reducing the intensity of the immunosuppressive treatment. If there is no response, treatment for polymorphic subtypes is rituximab monotherapy. For more well-defined lymphoma types, such as DLBCL, treatment in addition to modifying the immunosuppressive therapy should consist of CHOP-based therapy with rituximab, according to regular guidelines.