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Article

The histiocytosis Erdheim-Chester disease is an inflammatory myeloid neoplasm.

Expert review of clinical immunology - 1 Jan 2015

Haroche Julien, Cohen-Aubart Fleur, Charlotte Frédéric, Maksud Philippe, Grenier Philippe A, Cluzel Philippe, Mathian Alexis, Emile Jean-François, Amoura Zahir

Abstract excerpt

Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis, characterized by the infiltration of tissues by foamy CD68(+)CD1a(-) histiocytes. (99)Technetium bone scintigraphy revealing almost constant tracer uptake by the long bones is highly suggestive of ECD, and a 'hairy kidney' appearance on abdominal computed tomography scan is observed in about half of all ECD cases. CNS involvement is a strong...

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Identifiers

PMID: 26197238
DOI: 10.1586/1744666X.2015.1060857

Genes

ECD
BRAF
NRAS

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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