This Article Order Full text via Infotrieve Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by White, J. G. Articles by Clawson, C. C. Search for Related Content PubMed PubMed Citation Articles by White, J. G. Articles by Clawson, C. C.

American Journal of Pathology, Vol 96, 781-798, Copyright © 1979 by American Society for Investigative Pathology

REGULAR ARTICLES

The Chediak-Higashi syndrome: ring-shaped lysomomes in circulating monocytes

JG White and CC Clawson

The giant inclusion anomaly of leukocytes from three patients with the Chediak-Higashi syndrome (CHS) has been reexamined by the ultrastructural and cytochemical techniques placing emphasis on the monocyte. Contrary to previous assumptions that all CHS leukocytes shared a common form of giant granule with similar mechanisms of formation, it is shown that CHS monocytes contain ring-shaped organelles as their principle manifestation of the disease. These granules consist of a double membrane surrounding a segment of cytoplasm, which may or may not be sequestered from the remaining cytoplasm and which usually contains one or more normal-sized azurophilic granules. The material lying between the double membranes contains the specific reaction products for peroxidase and acid phosphatase. Therefore, the ring-shaped organelles share identity with the azurophilic granules and probably share a common origin with the primary lysosomes. The double-ring form of giant CHS organelle has also been found infrequently in neutrophils, eosinophils, and lymphocytes.