CHEDIAK-HIGASHI-SYNDROME - RING-SHAPED LYSOSOMES IN CIRCULATING MONOCYTES

  • 1 January 1979
    • journal article
    • research article
    • Vol. 96  (3) , 781-+
Abstract
The giant inclusion anomaly of leukocytes from 3 patients with Chediak-Higashi syndrome (CHS) was re-examined by ultrastructural and cytochemical techniques with emphasis on the monocyte. Contrary to previous assumptions that all CHS leukocytes share a common form of giant granule with similar formation mechanisms, CHS monocytes contain ring-shaped organelles as their principle manifestation of disease. These granules consisted of a double membrane surrounding a cytoplasmic segment which may or may not be sequestered from the remaining cytoplasm and which usually contained 1 or more normal-sized azurophilic granules. Material between the double membranes contained specific reaction products for peroxidase and acid phosphatase. Ring-shaped organelles share identity with the azurophilic granules and probably share a common origin with primary lysosomes. The double-ring form of giant CHS organelle also was found infrequently in neutrophils, eosinophils and lymphocytes.