Granulocyte chemotaxis in the Chediak-Higashi syndrome of mink.

Studies of granulocyte chemotaxiswere Higashi mink generated normal performed in mink with the Chediakamounts of chemotactic factors. The Higashi syndrome. In vivo migration cellular defect in leukocyte chemotaxis of leukocytes to an inflammatory site in mink is comparable to that observed was reduced in the affected animals. in humans with the Chediak-Higashi In vitro studies documented a consistsyndrome and may contribute to the ent early impairment in the chemotacincreased susceptibility to infection in tic response of Chediak-Higashi mink this disease. granulocytes. Serum from ChediakT HE CHEDIAK-HIGASHI SYNDROME (CHS) is a rare disorder inherited as an autosomal recessive trait and characterized by partial oculocutaneous albinism and giant lysosomal granules’ in leukocytes and other cells. Following its description in humans,2 this disease was also found to occur in mink, cattle,3 and mice.4 In man, the most prominent clinical feature has been recurrent, severe, and often fatal pyogenic infections.57 An increased susceptibility to bacterial infections has also been noted in CHS mink,7 although the most frequent problem is Aleutian disease, a chronic viral infection.6’7 Recent studies8 have demonstrated a cellular defect in granulocyte chemotaxis in humans with the Chediak-Higashi syndrome. The current report describes in vivo Rebuck skin window and in vitro Boyden chamber studies of leukocyte chemotaxis in CHS mink and documents a cellular chemotactic defect comparable to that observed in humans with CHS.