Abnormal Kinetics of Degranulation in Chronic Granulomatous Disease

Abstract

The rate of degranulation by neutrophils from three male patients with X-linked chronic granulomatous disease was studied with use of non-ingestable heat-aggregated human IgG as the phagocytic stimulus. Both the spontaneous and the stimulated release of two lysosomal enzymes (acid phosphatase and β-glucuronidase) was measured at various times. The difference between these two measurements, called the net release, was compared to that from 25 control subjects. For the first 15 minutes after phagocytic stimulation the net release of lysosomal enzymes was significantly reduced in neutrophils from the patients and their mothers in comparison to controls. By 30 minutes, the net release became normal. The total content of acid phosphatase and β-glucuronidase in polymorphonuclear leukocytes from patients was normal. These data suggest impaired lysosomal degranulation during the early stages of phagocytosis in X-linked chronic granulomatous disease. (N Engl J Med 291:332–337, 1974)