title: Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society.
abstract: Pathologists and pediatric hematologist/ oncologists of the World Health Organization's Committee on Histiocytic/Reticulum Cell Proliferations and the Reclassification Working Group of the Histiocyte Society present a classification of the histiocytic disorders that primarily affect children. Nosology, based on the lineage of lesional cells and biological behavior, is related to the ontogeny of histiocytes (macrophages and dendritic cells of the immune system). Dendritic cell-related disorders of varied biological behavior are dominated by Langerhans cell histiocytosis, but separate secondary proliferations of dendritic cells must be differentiated. Juvenile xanthogranuloma represents a disorder of dermal dendrocytes, another dendritic cell of skin. The hemophagocytic syndromes are the most common of the macrophage-related disorders of varied biological behavior. Guidelines for distinguishing the exceedingly rare malignant diseases of histiocytes from large cell lymphomas through the use of a battery of special studies are provided.
authors.affiliation: National Institutes of Health, Rocky Mountain Laboratories, Laboratory of Persistent Viral Diseases, Hamilton, MT 59840-2999, USA. Blaise_Favara@NIH.GOV
authors.forename: B E
authors.initials: BE
authors.lastname: Favara
authors.forename: A C
authors.initials: AC
authors.lastname: Feller
authors.forename: M
authors.initials: M
authors.lastname: Pauli
authors.forename: E S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: L M
authors.initials: LM
authors.lastname: Weiss
authors.forename: M
authors.initials: M
authors.lastname: Arico
authors.forename: P
authors.initials: P
authors.lastname: Bucsky
authors.forename: R M
authors.initials: RM
authors.lastname: Egeler
authors.forename: G
authors.initials: G
authors.lastname: Elinder
authors.forename: H
authors.initials: H
authors.lastname: Gadner
authors.forename: M
authors.initials: M
authors.lastname: Gresik
authors.forename: J I
authors.initials: JI
authors.lastname: Henter
authors.forename: S
authors.initials: S
authors.lastname: Imashuku
authors.forename: G
authors.initials: G
authors.lastname: Janka-Schaub
authors.forename: R
authors.initials: R
authors.lastname: Jaffe
authors.forename: S
authors.initials: S
authors.lastname: Ladisch
authors.forename: C
authors.initials: C
authors.lastname: Nezelof
authors.forename: J
authors.initials: J
authors.lastname: Pritchard
mesh_terms: D019070:Cell Lineage; D002648:Child; D003713:Dendritic Cells / Q000473:pathology / Q000502:physiology; D006644:Histiocytes / Q000473:pathology / Q000502:physiology; D054747:Histiocytic Sarcoma / Q000145:classification*; D015614:Histiocytosis / Q000145:classification*; D006646:Histiocytosis, Langerhans-Cell / Q000145:classification; D015616:Histiocytosis, Non-Langerhans-Cell / Q000145:classification; D006801:Humans; D008264:Macrophages / Q000473:pathology / Q000502:physiology; D014972:Xanthogranuloma, Juvenile / Q000145:classification