Chediak-Higashi syndrome: immunological responses to Epstein-Barr virus studies in gene heterozygotes.

Abstract

Immunologic studies were performed in five fathers and nine mothers of patients with Chediak-Higashi Syndrome (CHS). Antibody response to Epstein-Barr virus capsid antigen was higher than in normal controls. Antibodies to diffuse component of the early antigen were not detected and serum antibodies to the restricted component of the early antigen were observed in 64% of the subjects studied. Low natural killer activity and increased proportions of OKT8 positive cells were increased. These data indicate that immunologic alterations similar to those seen in CHS patients can be observed in their asymptomatic parents.