title: Giant cytoplasmic granules in Langerhans cells of Chediak-Higashi syndrome.
abstract: Giant membrane-bound cytoplasmic granules were found in the epidermal Langerhans cells of a patient with the Chediak-Higashi syndrome. These cells also contained normal-appearing Birbeck granules. The giant granules had a granular or sometimes globular internal structure; they are believed to derive from fusion of lysosomes or some portion of Birbeck granules. It is unclear whether this morphologic change in Langerhans cell interferes with their antigen-presenting function; it may be, in part, responsible for the frequent infections seen in patients with Chediak-Higashi syndrome that are otherwise more clearly related to the abnormalities in neutrophils and lymphocytes. The Langerhans cell is another cellular type in Chediak-Higashi syndrome in which giant cytoplasmic granules are found.
authors.affiliation: Department of Histology, Faculty of Medicine, Universidad, Nacional Autónoma de México.
authors.forename: J
authors.initials: J
authors.lastname: Carrillo-Farga
authors.forename: G
authors.initials: G
authors.lastname: Gutiérrez-Palomera
authors.forename: R
authors.initials: R
authors.lastname: Ruiz-Maldonado
authors.forename: A
authors.initials: A
authors.lastname: Rondán
authors.forename: S
authors.initials: S
authors.lastname: Antuna
mesh_terms: D001706:Biopsy; D002609:Chediak-Higashi Syndrome / Q000473:pathology*; D003594:Cytoplasmic Granules / Q000648:ultrastructure*; D006801:Humans; D007223:Infant; D007801:Langerhans Cells / Q000648:ultrastructure*; D008297:Male; D008544:Melanocytes / Q000473:pathology / Q000648:ultrastructure; D008854:Microscopy, Electron; D012867:Skin / Q000473:pathology / Q000648:ultrastructure