Neutrophilic function in animals with the Chediak-Higashi syndrome.

T HE CHEDIAK-HIGASHI SYNDROME (C-HS) has been reported in three species: man,’ mink,23 and cattle.4 This syndrome has recently been reviewed and the general characteristics of the syndrome will not he considered here.5 In all three species there is an increased susceptibility to chronic infectious diseases. Children with this condition have had repeated cutaneous infections, unexplained pyrexic episodes, recurrent oral ulcers, and pneumonia. Staph ylococcus aureus has been isolated repeatedly from the children. Epidemiologic studies have shown C-HS mink to be more susceptible than non C-HS mink to Staphylococcus aureus,6 Pasteurelia multocida and Corynebacterium S7).5 as well as Aleutian disease virus.7 All postmortem examinations of C-HS cattle revealed disseminated bacterial abscesses caused by various microorganisms.5 No investigator has demonstrated a difference in the immune mechanism when C-HS and non-C-HS individuals of the three species were compared. Since the turn of the century, the phagocytic theory of host resistance to infection has been accepted. The phagocytes are considered to be one of the principal agencies of the host in defense against microbial invaders. Neutrophils, as well as other granule-forming cells of C-HS individuals, contain giant granules. The granules of the neutrophil and other leukocytes have been shown to be lysosomes.8 In view of the apparent normality of the immunologic defenses and the lysosomal abnormality present in the neutrophil, it was of interest to search for a chemical or functional defect of this cell which might account for the increased susceptibility of C-HS individuals to microbial invasion.