“…Death may occur at an early age due to infection or a lymphoma-like accelerated phase which may be of Tcell origin (Argyle et aZ., 1982) or rather a lymphohistiocytic proliferation related to EBV infection (Rubin et al, 1985). Other cellular processes that have been shown to be abnormal include absent or decreased natural killer (NK) activity (Haliotis et aZ., 1980;Klein et al, 1980;Merino et al, 1983;Roder and Duwe, 1979), altered responsiveness to EBV infection (Merino et al, 1986), decreased antibody-dependent cell-mediated cytotoxicity (ADCC) (Klein et aL, 1980), and altered neutrophil digestive capabilities (Quie and Cates, 1977). Beige (bg) mice have many of these features, and several groups have proposed that the murine bg mutation is analogous to the mutation in patients with CHS (Brandt et aL, 1975;Lutzner et al, 1966;Windhorst and Padgett, 1973).…”