AIDS-related lymphomas: Primary central nervous system lymphoma
- Lawrence D Kaplan, MD
Lawrence D Kaplan, MD
- Clinical Professor of Medicine
- University of California, San Francisco
- James L Rubenstein, MD, PhD
James L Rubenstein, MD, PhD
- Professor of Medicine, Division of Hematology/Oncology
- University of California, San Francisco
Infection with human immunodeficiency virus (HIV) predisposes individuals to the development of neoplasms. There are currently four acquired immune deficiency syndrome (AIDS)-defining malignancies: Kaposi's sarcoma, non-Hodgkin lymphoma (NHL) of high-grade pathologic type and of B cell or unknown immunologic phenotype, primary central nervous system lymphoma (PCL), and invasive cervical carcinoma. AIDS-related NHL is generally divided into three types: systemic NHL, PCL, and the primary effusion ("body cavity") lymphomas [1,2]. More unusual types, such as plasmablastic lymphoma, also occur. These disorders are primarily encountered in patients with more advanced HIV infection, with a CD4 count that is usually below 100/microL . (See "AIDS-related lymphomas: Epidemiology, risk factors, and pathobiology" and "HIV infection and malignancy: Epidemiology and pathogenesis", section on 'Epidemiology'.)
The major issues relating to AIDS-related PCL will be reviewed here. Reviews of the diagnosis and treatment of PCL not associated with HIV infection are presented separately. Although central nervous system (CNS) involvement also occurs in AIDS-related systemic lymphoma in the form of secondary spread of the tumor to the meninges, the disease is limited to the CNS in PCL. Evaluation of the CNS in patients with AIDS-related systemic lymphoma is discussed separately. (See "Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma" and "Treatment and prognosis of primary central nervous system lymphoma" and "AIDS-related lymphomas: Clinical manifestations, diagnosis, and staging of systemic lymphoma".)
The pathogenesis of PCL is strongly related to Epstein-Barr virus infection. This is described in more detail separately. (See "AIDS-related lymphomas: Epidemiology, risk factors, and pathobiology", section on 'Pathobiology'.)
PCL accounts for up to 15 percent of non-Hodgkin lymphomas (NHLs) in human immunodeficiency virus (HIV)-infected patients compared with only 1 percent of NHLs in the general population . The reported incidence of PCL in HIV-infected patients is 2 to 6 percent (at least 1000 times higher than in the general population)  and has been as high as 10 percent in autopsy series . The most common histology was diffuse large B cell lymphoma (immunoblastic variant) . (See "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma".)
The incidence of PCL increases with prolonged survival from human immunodeficiency virus (HIV)-1 infection and requires a more severe degree of immunosuppression than most other acquired immune deficiency syndrome (AIDS)-related complications; the CD4 counts in affected patients are generally less than 50 cells/microL [6-8]. Thus, PCL is rarely an initial AIDS-defining illness.
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