Encyclopedia of AIDS

Living Edition
| Editors: Thomas J. Hope, Douglas Richman, Mario Stevenson

AIDS-Related Primary Central Nervous System Lymphoma

  • Jan Davidson-Moncada
  • Thomas S. Uldrick
Living reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9610-6_16-1


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 pathological and clinical overlap, AR-PCNSL is distinguished by its strong association with immunosuppression and viral etiology.


Primary central nervous system lymphoma (PCNSL) is a rare extra-nodal non-Hodgkin lymphoma, which accounts for approximately 3–5 % of primary brain tumors. Prior to effective therapy for HIV, a large proportion of cases of PCNSL, especially in younger individuals, occurred in severely...


Cerebral Spinal Fluid Primary Central Nervous System Lymphoma Leptomeningeal Dissemination Primary Central Nervous System Lymphoma Patient Systemic DLBCL 
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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Medical Oncology BranchCenter for Cancer Research, National Cancer Institute (NCI)BethesdaUSA
  2. 2.Retroviral Disease Section, HIV and AIDS Malignancy BranchCenter for Cancer Research, National Cancer Institute (NCI)BethesdaUSA