It has long been recognized that patients with both inherited and acquired abnormalities of cellular immunity have an increased incidence of non-Hodgkin's lymphoma. The incidence of lymphoproliferative disorders in patients with primary immunodeficiency diseases, such as ataxia telangiectasia or the Wiscott-Aldrich syndrome, is approximately 100 times greater than expected; NHL in allograft recipients has an incidence 60 times that of the general population. In a similar way to these patients, individuals infected with HIV have defective cellular immunity and are thus at risk of developing B-cell malignancy, which is recognized as an AIDS-defining diagnosis. Both systemic and cerebral NHL occur in this population with the relative risk being approximately 100-fold higher than that expected in the general population.
The introduction of combination highly active anti-retroviral treatment (HAART) in the late 1990s has led to a reduction in AIDS-defining illnesses, including NHL. Adjusted incidence rates show a decline in incidence of AIDS-related lymphoma (ARL) from 1992 to 1996, and from 1997 to 1999, with a relative risk ratio of 0.58. This decline is most marked for cerebral lymphoma but is also significant for systemic disease. There remains, however, a significant risk among patients on HAART, and a higher risk in those who do not have access to HAART, either because of economic restrictions or because they are unaware of their HIV status. Overall, it is estimated that approximately 10% of HIV-infected individuals will develop NHL.
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