Several studies suggest that the Epstein-Barr virus (EBV) is etiologic
ally linked to Hodgkin's disease (HD). This study was undertaken to ex
amine the role of EBV in familial HD (FHD). Among 60 FHD patients from
27 families with two or more cases per family, we tested available pa
raffinized tumor tissues from 46 cases by in situ hybridization for EB
V-encoded RNA (EBER1) expression. Thirteen of 46 FHD patients (28%) ha
d EBER1 expressed in the Reed-Sternberg cells. Concordance rate of EBV
positivity was evaluated among 34 first-degree related pairs from 17
families for which both cases had available paraffinized tumor tissues
. Only two of 17 pairs were concordant for EBER1 positivity, There was
no excess of positive concordance (P=.18). Serologically, FHD patient
s had higher geometric mean antibody titers (GMTs) to the viral capsid
antigen (VCA) and early antigen D (EA-D). There was no difference in
seroprevalence between patients and control groups, nor was there conc
ordance in elevated serology among 15 pairs of first-degree related FH
D cases, Young adult unaffected family members (UFM) may not react to
EBV in the same way as the general population as evidenced by the lowe
r titer of VCA, although not statistically significant, and significan
tly lower titers of EA-D, compared with age-matched controls, While EB
V might have some role in a subset of HD, lack of concordance of EBER1
expression and EBV serology among the FHD cases in the same family su
ggest that EBV does not play an important role in FHD. (C) 1996 by The
American Society of Hematology.