Article
Xanthomatous Inflammatory Infiltrate Involving the Spleen: An Unusual Presentation of Erdheim-Chester Disease and Review of the Literature.
The American journal of case reports - 4 Jun 2021
Di Stefano Gioia, Granai Massimo, Giudici Francesco, Roselli Giuliana, Lazzi Stefano, Santi Raffaella
Abstract excerpt
BACKGROUND Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by foamy histiocytes, Touton-like giant cells, and fibrosis, typically affecting the diaphyseal and metaphyseal region of the long bones but that can involve any organ or tissue. ECD is usually associated with the BRAF V600E mutation or with other molecular mutations inserted in the MAPK cascade. CASE REPORT...
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