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Cardiac and pleuropulmonary involvement in Erdheim-Chester disease without bone lesions: A case report.

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.

SUBMITTER: Kanza RE 

PROVIDER: S-EPMC8688944 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature

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Cardiac and pleuropulmonary involvement in Erdheim-Chester disease without bone lesions: A case report.

Kanza Rene Epunza RE   Houle Olivier O   Simard Pierre-Luc PL   St-Gelais Jonathan J   Raymond Catherine C  

Radiology case reports 20211216 3

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 <i>BRAF</i> 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. ...[more]