Ultrastructures of Reilly bodies (metachromatic granules) in the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI). A histochemical study.
The leukocytes from an 8-year-old white girl having features of the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI) contained large granular inclusions (Reilly granules). Light and electron microscopy showed that these granules had characteristics of those seen in several other types of mucopolysaccharidoses, including Morquio's syndrome, which are of a different structure than those of other types of mucopolysaccharidosis. Similar granules were seen in the platelets, a finding which, so far as we know, has never been reported before. The granules contained an acid mucosubstance demonstrated by cytochemical studies.
D001792:Blood Platelets / Q000648:ultrastructure; D002648:Child; D003594:Cytoplasmic Granules / Q000473:pathology / Q000648:ultrastructure*; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007962:Leukocytes / Q000473:pathology / Q000648:ultrastructure*; D009083:Mucopolysaccharidoses / Q000473:pathology*; D009087:Mucopolysaccharidosis VI / Q000473:pathology*