Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia

Abstract

Non-Hodgkin's lymphoma (NHL) is the most common human immunodeficiency virus (HIV)-associated malignancy in hemophiliacs. We studied the incidence and clinicopathologic features of NHL in 3,041 hemophiliacs followed at 18 US Hemophilia Centers between 1978 and 1989. Of the 1,295 (56.6%) who were HIV(+), 253 (19.5%) developed acquired immunodeficiency syndrome (AIDS), of whom 14 (5.5%) developed NHL. Three NHL occurred in HIV(-) hemophiliacs, for a 36.5-fold greater risk in HIV(+) than HIV(-) hemophiliacs (P < .001). The NHL incidence rate was 29-fold greater than in the US population by Surveillance, Epidemiology, and End Results (SEER) estimates (P < .001). Between 0 and 4 lymphomas have been observed per year between 1978 and 1989. At presentation 13 (92.9%) of the HIV(+) NHL were extranodal. Ten were stage IV, 1 stage II, and 3 stage IE. Ten (71.4%) were high-grade, 3 (21.4%) intermediate-grade, and 1 (7.1%) was a low-grade B-cell lymphoma. Epstein-Barr virus (EBV) DNA was detected in 36% by in situ hybridization, including one central nervous system (CNS) lymphoma. The mean CD4 cell count at NHL diagnosis was 64/mm3, the mean latency from initial HIV infection was estimated to be 59 months, and the median survival was 7 months. The incidence of basal cell carcinoma in HIV(+) hemophiliacs was 18.3-fold greater than in HIV(-) hemophiliacs (P < .001) and 11.4-fold greater than in the US population (P < .001). In conclusion, incidence rates of NHL and basal cell carcinoma in HIV(+) hemophiliacs are significantly increased over rates in HIV(-) hemophiliacs and over rates in the US population. Clinicopathologic presentation of NHL in HIV(+) hemophiliacs is similar to that in HIV(+) homosexual men.

Molecular aetiology and pathogenesis of basal cell carcinoma

“…Seemingly in contradiction to the lack of an increase in the incidence of BCC in organ recipients, people suffering from acquired immune deficiency syndrome (AIDS) have shown an elevated risk for the development of BCC. 149,150 Human immunodeficiency virus (HIV) patients with BCC more frequently show blue eyes, blond hair, family history and extensive prior sun exposure. 151 The pigmentation phenotype is probably an independent risk factor that is added to the increased risk of BCC conferred by the immunosuppression.…”

Genetic heterogeneity of AIDS‐related small non‐cleaved cell lymphoma

“…Non-Hodgkin's lymphomas (NHL) represent a major complication of HIV infection and constitute an AIDS-defining illness (Beral et al, 1991;Carbone et al, 1991;Levine, 1993;. Since the outbreak of the AIDS epidemic, the incidence of AIDS-related NHL (AIDS-NHL) has been rising steadily and, at present, AIDS-NHL are the most frequent cancer in AIDS patients who have acquired the infection parenterally (Beral et al, 1991;Levine, 1993;Ragni et al, 1993;. AIDS-NHL consistently derive from B cells, tend to be diffusely aggressive, and display a number of clinical differences when compared to NHL of similar histology arising in the immunocompetent host, including a high frequency of systemic symptoms, a poor prognosis, and frequent involvement of extranodal sites (Levine, 1993).…”

Malignancy in patients with haemophilia: a review of the literature

“…Results on 3041 US patients with haemophilia were published by Ragni et al in 1993 as part of the Haemophilia Malignancy Study [18]. Three cases of leukaemia were reported (1 HIV positive, 2 HIV negative) however no additional data was provided.…”

“…The final report from the US Haemophilia Malignancy Study identified 30 solid tumours (basal cell carcinoma = 10, testicular cancer = 5, melanoma = 4, head and neck cancer = 4, prostate cancer = 2, HCC = 2, lung cancer = 1, colon cancer = 1, bladder cancer = 1) [18]. Age ranges were not reported.…”