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

Margaret V. Ragni, Steven H. Belle, Ronald A. Jaffe, Sandra L. Duerstein, Debra C. Bass, Campbell W. McMillan, E. W. Lovrien, Louis M. Aledort, C. T. Kisker, Sally P. Stabler, W. Keith Hoots, Margaret W. Hilgartner, Joan Cox-Gill, George R. Buchanan, Natalie L. Sanders, Doreen B. Brettler, Lloyd E. Barron, Jonathan C. Goldsmith, Bruce Ewenstein, Kenneth J. SmithDavid Green, Joseph E. Addiego, Lawrence A. Kingsley

Research output: Contribution to journalArticle

66 Scopus citations

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 < .0001) 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.

Original languageEnglish (US)
Pages (from-to)1889-1897
Number of pages9
JournalBlood
Volume81
Issue number7
DOIs
StatePublished - Jan 1 1993

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Ragni, M. V., Belle, S. H., Jaffe, R. A., Duerstein, S. L., Bass, D. C., McMillan, C. W., Lovrien, E. W., Aledort, L. M., Kisker, C. T., Stabler, S. P., Hoots, W. K., Hilgartner, M. W., Cox-Gill, J., Buchanan, G. R., Sanders, N. L., Brettler, D. B., Barron, L. E., Goldsmith, J. C., Ewenstein, B., ... Kingsley, L. A. (1993). Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia. Blood, 81(7), 1889-1897. https://doi.org/10.1182/blood.v81.7.1889.1889