31 The 'Impotent Neutrophil' Syndrome

Abstract

Leukocyte function was studied in a 2–2,5-year-old Negro male with the history of repeated abscesses, cervical and inguinal adenitis, recurrent pneumonia, seborrheic eczema of the scalp, hepatosplenomegaly and anemia. An appropriate leukocytosis with neutrophilia occurred with each infection, absolute neutrophil counts ranging from 3,900 to 24,500/mm3. IgG, IgA and IgM globulins were present in increased amounts. Lymph node biopsy contained caseating granuloma. Skin tests and cultures for all types of acid fast bacilli and fungi were repeatedly negative. These findings are characteristic of the syndrome of chronic granulomatous disease of childhood. There was a normal flux of leukocytes into Rebuck skin windows and exudate fluid. Leukocytes appeared normal with alkaline phosphatase, peroxidase and Wright's stain and were normally vacuolated after bacterial phagocytosis. Normal leukocyte motility and phagocytosis followed by degranulation were seen with phase microscopy. Decreased bactericidal activity of the patient's leukocytes during in vitro incubation with Staphylococcus aureus and Aerobacter aerogenes was repeatedly demonstrated in the presence of the patient's serum or normal serum. To determine the nature of this defect a citric acid extract of the patient's leukocyte granules was compared with control specimens and decreased bactericidal activity was found. The deficient or abnormal phagocytin activity of the patient's leukocytes, demonstrated by this study, could account for his clinical syndrome. (SPR)