Clinical features and treatment strategies for post-transplant and iatrogenic immunodeficiency-associated lymphoproliferative disorders.

A specific category termed immunodeficiency-associated lymphoproliferative disorders (LPD) exists in the 2016 revised WHO classification concerning lymphoid neoplasms. This category is defined by etiology and includes LPD developing in association with organ transplantation or immunosuppressive/immunomodulatory agents including methotrexate. The functional mechanism is chiefly explained by the autonomous proliferation of Epstein-Barr virus (EBV)-infected lymphocytes induced by host-immune suppression. This category ranges from reactive lymphocyte hyperplasia to monomorphic lymphoma. Its clinical behavior varies depending on host immunity and pathological features; pathological confirmation by biopsy is thus important for deciding treatment strategies. Owing to the spontaneous regression observed in some patients, uniform chemotherapy is not recommended. The main initial treatment options include the reduction in immunosuppressive drugs, immunotherapy with the anti-CD20 antibody rituximab, chemotherapy, or a combination of these. Other novel treatments such as adoptive immunotherapy with EBV-specific cytotoxic T cells, could be an alternative for relapsed/refractory diseases in clinical trials.