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Cardiac and pleuropulmonary involvement in Erdheim-Chester disease without bone lesions: A case report
Rene Epunza Kanza1,2, Olivier Houle2, Pierre-Luc Simard3
1Department of Radiology, Chicoutimi Hospital, 305 Rue Saint-Vallier, Saguenay, QC G7H5H6, Canada.
Abstract:
Erdheim-Chester disease is a rare multisystemic non-Langerhans histiocytosis characterized by histiocytes that stain positive for CD68 and negative for CD1a. Skeletal involvement is reported to be present in up to 96% cases and BRAF mutation in about half of the cases. Here, we report a patient with an unusual longstanding BRAF-negative Erdheim-Chester disease without bone lesions who developed pleuropulmonary and cardiac involvement.
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