title: Ceroid pigment deposition in circulating blood monocytes and T lymphocytes in Hermansky-Pudlak syndrome: an ultrastructural study.
abstract: An electron microscopic study of peripheral leukocytes obtained from a 39 year old woman with Hermansky-Pudlak syndrome was performed. Ceroid pigment granules were found within the lysosomes in 3.5% of monocytes and 5.4% of lymphocytes. Infrequently, pigment granules were also found in the parallel tubular arrays of lymphocytes. The lymphocytes containing ceroid pigment granules were confirmed to be T cells by immunoelectron microscopy. It was speculated that intralysosomal accumulation of ceroid pigment granules in Hermansky-Pudlak syndrome may be due to lysosomal dysfunction.
authors.affiliation: Department of Pathology, Iwate Prefectural Central Hospital, Morioka, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Sakuma
authors.forename: N
authors.initials: N
authors.lastname: Monma
authors.forename: R
authors.initials: R
authors.lastname: Satodate
authors.forename: T
authors.initials: T
authors.lastname: Satoh
authors.forename: R
authors.initials: R
authors.lastname: Takeda
authors.forename: S
authors.initials: S
authors.lastname: Kuriya
mesh_terms: D000328:Adult; D016115:Albinism, Oculocutaneous / Q000276:immunology / Q000473:pathology*; D002566:Ceroid / Q000097:blood*; D005260:Female; D006801:Humans; D016253:Microscopy, Immunoelectron; D009000:Monocytes / Q000737:chemistry / Q000648:ultrastructure*; D013601:T-Lymphocytes / Q000737:chemistry / Q000648:ultrastructure*
chemical_list: D002566:Ceroid