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

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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
StatePublished - Apr 1 1993

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Hemophilia A
Viruses
Non-Hodgkin's Lymphoma
Acquired Immunodeficiency Syndrome
HIV
Neoplasms
Basal Cell Carcinoma
Incidence
Cells
Lymphoma
Population Surveillance
Epidemiology
B-Cell Lymphoma
Virus Diseases
CD4 Lymphocyte Count
Neurology
Human Herpesvirus 4
Population
In Situ Hybridization
Central Nervous System

ASJC Scopus subject areas

  • Hematology

Cite this

Ragni, M. V., Belle, S. H., Jaffe, R. A., Duerstein, S. L., Bass, D. C., McMillan, C. W., ... Kingsley, L. A. (1993). Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia. Blood, 81(7), 1889-1897.

Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia. / Ragni, Margaret V.; Belle, Steven H.; Jaffe, Ronald A.; Duerstein, Sandra L.; Bass, Debra C.; McMillan, Campbell W.; Lovrien, E. W.; Aledort, Louis M.; Kisker, C. T.; Stabler, Sally P.; Hoots, W. Keith; Hilgartner, Margaret W.; Cox-Gill, Joan; Buchanan, George R.; Sanders, Natalie L.; Brettler, Doreen B.; Barron, Lloyd E.; Goldsmith, Jonathan C.; Ewenstein, Bruce; Smith, Kenneth J.; Green, David; Addiego, Joseph E.; Kingsley, Lawrence A.

In: Blood, Vol. 81, No. 7, 01.04.1993, p. 1889-1897.

Research output: Contribution to journalArticle

Ragni, MV, Belle, SH, Jaffe, RA, Duerstein, SL, Bass, DC, McMillan, CW, Lovrien, EW, Aledort, LM, Kisker, CT, Stabler, SP, Hoots, WK, Hilgartner, MW, Cox-Gill, J, Buchanan, GR, Sanders, NL, Brettler, DB, Barron, LE, Goldsmith, JC, Ewenstein, B, Smith, KJ, Green, D, Addiego, JE & Kingsley, LA 1993, 'Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia', Blood, vol. 81, no. 7, pp. 1889-1897.
Ragni MV, Belle SH, Jaffe RA, Duerstein SL, Bass DC, McMillan CW et al. Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia. Blood. 1993 Apr 1;81(7):1889-1897.
Ragni, Margaret V. ; Belle, Steven H. ; Jaffe, Ronald A. ; Duerstein, Sandra L. ; Bass, Debra C. ; McMillan, Campbell W. ; Lovrien, E. W. ; Aledort, Louis M. ; Kisker, C. T. ; Stabler, Sally P. ; Hoots, W. Keith ; Hilgartner, Margaret W. ; Cox-Gill, Joan ; Buchanan, George R. ; Sanders, Natalie L. ; Brettler, Doreen B. ; Barron, Lloyd E. ; Goldsmith, Jonathan C. ; Ewenstein, Bruce ; Smith, Kenneth J. ; Green, David ; Addiego, Joseph E. ; Kingsley, Lawrence A. / Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia. In: Blood. 1993 ; Vol. 81, No. 7. pp. 1889-1897.
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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.",
author = "Ragni, {Margaret V.} and Belle, {Steven H.} and Jaffe, {Ronald A.} and Duerstein, {Sandra L.} and Bass, {Debra C.} and McMillan, {Campbell W.} and Lovrien, {E. W.} and Aledort, {Louis M.} and Kisker, {C. T.} and Stabler, {Sally P.} and Hoots, {W. Keith} and Hilgartner, {Margaret W.} and Joan Cox-Gill and Buchanan, {George R.} and Sanders, {Natalie L.} and Brettler, {Doreen B.} and Barron, {Lloyd E.} and Goldsmith, {Jonathan C.} and Bruce Ewenstein and Smith, {Kenneth J.} and David Green and Addiego, {Joseph E.} and Kingsley, {Lawrence A.}",
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AU - Ragni, Margaret V.

AU - Belle, Steven H.

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AU - Duerstein, Sandra L.

AU - Bass, Debra C.

AU - McMillan, Campbell W.

AU - Lovrien, E. W.

AU - Aledort, Louis M.

AU - Kisker, C. T.

AU - Stabler, Sally P.

AU - Hoots, W. Keith

AU - Hilgartner, Margaret W.

AU - Cox-Gill, Joan

AU - Buchanan, George R.

AU - Sanders, Natalie L.

AU - Brettler, Doreen B.

AU - Barron, Lloyd E.

AU - Goldsmith, Jonathan C.

AU - Ewenstein, Bruce

AU - Smith, Kenneth J.

AU - Green, David

AU - Addiego, Joseph E.

AU - Kingsley, Lawrence A.

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N2 - 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.

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