title: Establishment of a Langerhans cell histiocytosis lesion cell line with dermal dendritic cell characteristics.
abstract: A cell line named PRU-1, derived from a Langerhans cell (LC) histiocytosis (LCH) skull lesion of a 7-year-old boy, was established and characterized. PRU-1 is an adherent spindle-shaped cell line that shows no Birbeck granules on electron microscopy. Flow cytometric analysis of cells collected from the early seventh passage showed no LC phenotypes of CD1a and S100 protein. Immunostaining of PRU-1 cells also revealed no expression of LC markers but showed expression of CD11c, CD54 (ICAM-1) and CD68, which was also observed in some peripherally located cells of the original LCH lesion. The PRU-1 cells stained positive for factor XIIIa and negative for CD34, suggesting a dermal dendritic cell phenotype. Cytogenetic analyses revealed abnormalities such as 39,XY,-2,-4,-8,-12,-12,-14,add(18)(q21),20,+mar and 44,XY,-11,-14,add(18)(q21). TCRγ rearrangement in the PRU-1 cells was not amplified by PCR. Tumorigenicity was not proven by xenografting into SCID mice. A conditioned medium from PRU-1 culture induced the proliferation of peripheral blood lymphocytes as well as the activation of monocytes from a healthy donor into CD1a-positive LC-like cells. Because the phenotypic characteristics of PRU-1 differed from those of CD1a-positive abnormal LC-like cells (LCH cells), it was likely that the PRU-1 cells were derived from peripherally located cells of the LCH lesion rather than LCH cells. LCH has been regarded as a type of granulomatous neoplasm with several intermingled inflammatory cells and influenced by stimuli such as Merkel cell polyomavirus (MCPyV) infection or cigarette smoking. However, in the PRU-1 cells, MCPyV-DNA was not detected by PCR. Stromal cell-like PRU-1 cells are likely to produce some growth or differentiation factors, which may play important roles in LCH lesion formation, cell maintenance and LC-like cell induction.
authors.affiliation: Division of Molecular Pathology, Faculty of Medicine, Tottori University, Yonago, Tottori 683-8503, Japan.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Murakami
authors.affiliation: Inserm U507 and U1016, Institut Cochin, 75014 Paris, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Gogusev
authors.affiliation: University of Paris Descartes (Paris V), 75006 Paris, France.
authors.forename: Francis
authors.initials: F
authors.lastname: Jaubert
authors.affiliation: Department of Pathobiological Science and Technology, School of Health Science, Faculty of Medicine, Tottori University, Yonago 683-8503, Japan.
authors.forename: Michiko
authors.initials: M
authors.lastname: Matsushita
authors.affiliation: Division of Molecular Pathology, Faculty of Medicine, Tottori University, Yonago, Tottori 683-8503, Japan.
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Hayashi
authors.affiliation: Division of Hematology and Oncology, St. Marianna University School of Medicine Hospital, Kawasaki, Kanagawa 216-8511, Japan.
authors.forename: Ikuo
authors.initials: I
authors.lastname: Miura
authors.affiliation: Department of Pathology, Okayama University Hospital, Okayama 700-8530, Japan.
authors.forename: Takehiro
authors.initials: T
authors.lastname: Tanaka
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8530, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Oka
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8530, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
mesh_terms: D000818:Animals; D002460:Cell Line; D002648:Child; D017077:Culture Media, Conditioned; D003713:Dendritic Cells / Q000502:physiology* / Q000637:transplantation; D020405:Dermis / Q000473:pathology; D005260:Female; D006003:Glycogen / Q000378:metabolism; D064593:Heterografts; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D008214:Lymphocytes / Q000378:metabolism; D008297:Male; D016513:Mice, SCID; D010641:Phenotype
chemical_list: D017077:Culture Media, Conditioned; D006003:Glycogen