Abnormal erythrocyte and lymphocyte chemiluminescence in chronic granulomatous disease: Evidence for a generalized membrane defect

• Published in: Clinical immunology and immunopathology Vol. 38; no. 1; pp. 1 - 12
• Main Authors: Peerless, Allen G., Stiehm, E.Richard
• Format: Journal Article
• Language: English
• Published: San Diego, CA Elsevier Inc 1986; New York, NY Academic Press; Boston
• Subjects: Adolescent; Biological and medical sciences; blood cells; chemiluminescence; Child; Child, Preschool; chronic granulomatous disease; Cytotoxicity, Immunologic; Erythrocyte Membrane - metabolism; erythrocytes; Female; Granulomatous Disease, Chronic - blood; Hematologic and hematopoietic diseases; Humans; Infant; Luminescent Measurements; Luminol - pharmacology; lymphocytes; Lymphocytes - metabolism; Male; man; Medical sciences; membranes; NADH, NADPH Oxidoreductases - analysis; NADPH Oxidases; Neutrophils - metabolism; Other diseases. Hematologic involvement in other diseases; Zymosan - pharmacology; Pediatrics; Chronic; Red blood cell; Granulomatosis; Anomaly; Chemiluminescence; Hemopathy; Lymphocyte; Leukocyte disease
• ISSN: 0090-1229; 1090-2341
• DOI: 10.1016/0090-1229(86)90117-0

Concanavalin A (soluble)- and/or opsonized zymosan (particulate)-stimulated luminol-dependent chemiluminescence was demonstrated in normal human lymphocytes, erythrocytes, and in the membrane (ghost) fractions of polymorphonuclear neutrophils and erythrocytes. By contrast, seven patients with chronic granulomatous disease (five boys with the X-linked type and two female variants) had diminished luminescent responses to con A and/or opsonized zymosan in their lymphocytes, erythrocytes, erythrocyte membranes, as well as neutrophil membranes, suggesting generalization of the deficiency in the neutrophil oxidase system to other cells.