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American Journal of Pathology, Vol 122, 177-189, Copyright © 1986 by American Society for Investigative Pathology

REGULAR ARTICLES

Chediak-Higashi syndrome neutrophils are characterized by the absence of both normal azurophilic granules

BC West

Neutrophils from two Chediak-Higashi syndrome brothers were isolated, suspended in heparinized sucrose, lysed, and filtered. The granule-rich filtrate was centrifuged on a sucrose gradient (rho = 1.287-1.10 g/ml) at a mean force of 95,000g for 4 hours. The gradients contained one band at rho = 1.18 g/ml (band C) which was broader than normal and lacked normal bands A, rho = 1.22 g/ml, and B, rho = 1.20 g/ml. Gradient fractions were assayed for enzyme activities and protein. No marker enzymes identified densities normally occupied by bands A and B, and of the enzymes measured, only lysozyme showed peak activity with band C. Thus, only normal specific granules were present. Two azurophil granules, normally present and separable, were absent. Also identified was eosinophil granule peroxidase at rho = 1.24 g/ml (band E). Alkaline phosphatase, not a granule marker, was twice normal at the normal density, rho = 1.14-1.15 g/ml, consistent with an increase in unidentified membranes. A lysate gradient suggested that the giant azurophilic granules were rho = 1.25-1.27 g/ml. These neutrophils contain blue-grey or slate-grey giant granules, which are not truly azurophilic or basophilic, but should continue to be identified as azurophilic to conform to the convention making "azurophilic" and "peroxidase-positive" synonymous. The eosinophils contain normal eosinophil granules as well as giant inclusion granules. In contrast, neutrophils are deficient in both normal azurophilic granules.




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Copyright © 1986 by the American Society for Investigative Pathology.