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PMID: 474718 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

The Chédiak-Higashi syndrome: ring-shaped lysomomes in circulating monocytes.

The American journal of pathology ·Vol. 96 ·No. 3 ·1979-09-00 ·Pages 781-98

White JG, Clawson CC

Abstract

The giant inclusion anomaly of leukocytes from three patients with the Chédiak-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.

MeSH Terms
Acid Phosphatase/blood Adolescent Chediak-Higashi Syndrome/blood Humans Leukocytes/ultrastructure Lysosomes/ultrastructure Male Monocytes/enzymology,ultrastructure Organoids/ultrastructure Peroxidases/blood
Chemicals
Peroxidases Acid Phosphatase
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
White J G
Clawson C C
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38 references, click to expand
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Article Info
Journal
The American journal of pathology
Abbr.
Am J Pathol
ISSN
0002-9440
Published
1979-09-00
Pages
781-98
Language
English
Region
United States
NLM ID
0370502
PMCID
PMC2042404
Subset
IM
External Links
PubMed source PMC Full Text View journal details
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