DEFECTIVE POLYMORPHONUCLEAR LEUKOCYTE METABOLISM AND FUNCTION IN CANINE CYCLIC NEUTROPENIA

Abstract

Humans and grey collie dogs with cyclic neutropenia suffer from an increased rate of bacterial infection. Because of the previously described microanatomic abnormalities of lysosome formation found in the polymorphonuclear leukocytes (PMN) of dogs wth canine cyclic neutropenia, studies of these cells were undertaken. PMN from grey collie dogs had significant metabolic and functional abnormalities when compared with normal collie PMN. These included abnormally increased postphagocytic C1-glucose oxidation, decreased iodination of trichloroacetic acid-precipitable protein in the resting and phagocytizing state, decreased levels of intracellular myeloperoxidase and a bactericidal defect against a variety of bacteria [Staphylococcus aureus, Streptococcus faecalis and Escherichia coli]. Phagocytosis was normal. These abnormalities differed from those previously described in the PMN of patients with chronic granulomatous disease of childhood and the Chediak-Higashi syndrome and more closely resembled those seen in hereditary myeloperoxidase deficiency. Defective PMN function was demonstrated in a disease state previously believed to be a model only of periodic hematopoiesis.