Phagocytosis in Chronic Granulomatous Disease and the Chediak–Higashi Syndrome

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Abstract
Phagocytosis of emulsified paraffin oil containing oil red O by blood leukocytes from three male patients with chronic granulomatous disease was not abnormal, whereas the phagocytic rate and capacity of leukocytes from two patients with the Chediak-Higashi syndrome were doubled in relation to normal cells. The specific activities of acid hydrolases and peroxidase in whole homogenates or in oil-laden phagocytic vesicles isolated by flotation from normal leukocytes or those from patients with chronic granulomatous disease were similar. Specific activities of β-glucuronidase and peroxidase in vesicles obtained from leukocytes in the Chediak-Higashi syndrome were 56 to 67 per cent of normal, but activities of acid and alkaline phosphatases in the vesicles and of all the enzymes assayed in whole homogenates were normal. By direct quantification, therefore, the extent of degranulation by leukocytes of patients with chronic granulomatous disease is not impaired, whereas a possible selective degranulation defect has been detected in those of patients with the Chediak - Higashi syndrome.