Abstract
Primary central nervous system lymphoma (PCNSL) is a rare extra-nodal B-cell non-Hodgkin lymphoma, which arises in the brain, spinal cord, meninges, or eyes. In individuals infected with HIV, PCNSL occurs with advanced immunosuppression and low CD4 T-cell count. It is considered an AIDS-defining malignancy. Unlike PCNSL in immune-competent hosts, AIDS-related PCNSL (AR-PCNSL) is almost exclusively caused by a cancer-causing herpesvirus, Epstein–Barr Virus (EBV, also known as human herpesvirus-4). While PCNSL in immune-competent patients and AR-PCNSL have pathologic and clinical overlap, AR-PCNSL is distinguished by its strong association with immunosuppression and viral etiology.
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