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The appearance and worldwide spread of the human immunodeficiency virus (HIV) have been associated with a steep rise in the incidence of lymphoma in HIV-infected individuals, with lymphoma being an acquired immune deficiency syndrome (AIDS)-defining event in an infected person [1, 2]. The increased incidence is particularly in non-Hodgkin's lymphoma (NHL) and, to a lesser extent, in Hodgkin's disease. The increased lymphoma incidence is attributable to (i) a high rate of infection with oncogenic viruses such as Epstein–Barr virus (EBV) and human herpesvirus 8 (HHV8), previously known as Kaposi's sarcoma-associated herpesvirus (KSHV), (ii) a high rate of opportunistic infections leading to chronic stimulation of the immune system and (iii) failure of immune surveillance. The incidence of NHL is increased about 100-fold and of Hodgkin's disease (Hodgkin lymphoma) about sixfold.

There is also an increased incidence of NHL in patients with congenital or iatrogenic immune deficiency [3, 4]. Most iatrogenic cases follow immunosuppressive therapy for haemopoietic or solid organ transplantation or methotrexate treatment for autoimmune disease. Some immune deficiency diseases, e.g. ataxia-telangiectasia, have an increased incidence of lymphoma resulting from defective DNA repair rather than from the immune deficiency.

The lymphomas that are increased in incidence in HIV infection are summarized in Table 15.1, together with known aetiological factors for specific subtypes. Certain lymphomas show a very strong correlation with HIV positivity, specifically primary effusion lymphoma [5] and plasmablastic lymphoma of the oral cavity.

Table 15.1Lymphomas that are increased in incidence in HIV-infected individuals

Clinical features

The clinical features are those usually associated with lymphoma but the disease is more rapidly progressive and often presents at a more advanced stage. Extra-nodal disease is considerably more common than among other cases of lymphoma and unusual sites may be involved, e.g. oral cavity and pleural and peritoneal cavities. In addition to the usual clinical features of lymphoma, there are specific clinical features associated with individual lymphomas-intracerebral lymphoma, primary effusion lymphoma and mucosa-associated lymphoid tissue (MALT)-type lymphoma of the lungs. Cases of Burkitt's lymphoma and Hodgkin's disease often present with stage IV disease. Some patients have other ...

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