Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia.
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.
Ragni
M V
MV
University of Pittsburgh School of Medicine, Hemophilia Center of Western Pennsylvania 15219.
Belle
S H
SH
Jaffe
R A
RA
Duerstein
S L
SL
Bass
D C
DC
McMillan
C W
CW
Lovrien
E W
EW
Aledort
L M
LM
Kisker
C T
CT
Stabler
S P
SP
D000163:Acquired Immunodeficiency Syndrome / Q000453:epidemiology / Q000209:etiology / Q000401:mortality; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002280:Basal Cell Carcinoma / Q000453:epidemiology / Q000209:etiology / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D006679:HIV Seropositivity / Q000150:complications / Q000453:epidemiology; D006467:Hemophilia A / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality; D002836:Hemophilia B / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016483:Lymphoma, AIDS-Related / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000453:epidemiology / Q000209:etiology / Q000401:mortality