Morphometric granule analysis of the human neutrophil in a patient with Chediak-Higashi syndrome

Abstract

The neutrophils of Chediak-Higashi Syndrome (CHS) have a characteristic giant abnormal peroxidase-positive granule (PPG). This giant PPG has been thought to be the product of the fusion of all or part of the primary (azurophil) or PPG population with or without involvement of the secondary (specific) granules which are peroxidase-negative (PNG). Immunocytochemical studies have shown that the giant PPG contains lactoferrin a marker for the PNG, whereas biochemical studies of isolated CHS granules failed to localize PNG markers to the giant PPG band or show PNG depletion. Ultrastructural studies failed to show PNG depletion and considered PNG fusion to be rare. This study was undertaken to evaluate CHS neutrophil granules niorphometrically. Human peripheral blood neutrophils were collected from a male patient with CHS in heparin and fixed in 3% glutaraldehyde as a cell suspension and minced buffy coat. The cell suspension was stained for myeloperoxidase using 3,3'- diaminobenzidine (DAB) as substrate.