Abstract
Hemophiliac patients are at risk for the development of acquired immune deficiency syndrome. Asymptomatic hemophiliacs demonstrate immunoregulatory defects. The etiology of immune defects in this population is not well understood. In a group of 137 consecutively chosen hemophiliacs, EBV serology was examined.
Age related acquisition of EBV did not differ from normal controls, however, 66% of hemophiliacs have serologic patterns consistent with persistent or reactivation EBV infection. In 60 patients, immune studies revealed significant immunoregulatory abnormalities (OKT.4/T.8 1.08±.68 vs. 1.98±.79 in normal controls). Functional studies revealed significant depression of Con A responses (mean log10 CPM 4.704±.341 vs. 5.074±.140 in normal controls). We hypothesize that chronic antigenic stimulation by Factor VIII concentrate results in immunoregulatory disturbances and increased reactivation of EBV infection. Reactivated EBV infection contributes further to the immunoregulatory defects in patients with hemophilia.
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Sullivan, J., Brettler, D. & Levine, P. IMMUNOREGULATORY DEFECTS AND EPSTEIN-BARR VIRUS (EBV) IN HEMOPHILIA. Pediatr Res 18 (Suppl 4), 266 (1984). https://doi.org/10.1203/00006450-198404001-01038
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DOI: https://doi.org/10.1203/00006450-198404001-01038