AIDS-associated lymphomas and other cancers in individuals with haemophilia

Non-Hodgkin's lymphoma is the most common malignancy in individuals with haemophilia and human immunodeficiency virus (HIV) infection. Among individuals with haemophilia, lymphoma accounts for 5% of primary AIDS diagnoses, and it is the sixth most common primary AID-defining diagnosis in this group. At the time of AIDS-lymphoma diagnosis, the median age in haemophiliacs is 32 years, the mean CD4 count is 116 μL(-1) , and the mean time from seroconversion to development of AIDS-lymphoma is 8 years. The most common primary site of AIDS-lymphomas in haemophiliacs is the gastrointestinal tract, and the most common histological type is large cell lymphoma. EBV has been detected at a molecular level in half of all haemophilia AIDS-lymphomas and its presence is predictive of shorter survival than in those without detectable EBV. The median survival after diagnosis is 6 months, with or without treatment. An increasing proportion of these tumours are occurring as secondary AIDS diagnoses, associated with the longer duration of immunosuppression afforded by antiviral therapy. This paper reviews the natural history, immunological and viral features, clinical characteristics, and risk factors associated with AIDS-lymphoma in the haemophilia population.