Erdheim–Chester disease with cardiac involvement. Case report

Erdheim–Chester disease with cardiac involvement. Case report

ErdheimChester disease (ECD) is a rare non-Langerhans histiocytosis with multisystem inflammatory infiltrates consistent of monocytes/macrophages, reactive microenvironment and fibrotic fields. Cardiovascular involvement is one of the most frequent manifestations of ECD that can lead to life threati...

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Veröffentlicht in:Terapevtic̆eskii arhiv 2022-08, Vol.94 (7), p.897-902
Hauptverfasser: Latyshev, Vitalii D., Mershina, Elena А., Kostina, Irina E., Sinitsyn, Valentin Е., Lukina, Elena A.
Format: Artikel
Sprache:eng ; rus
Schlagworte:
erdheim–chester disease
mutation braf v600e
non-langerhans cell histiocytosis
trametinib
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Zusammenfassung:ErdheimChester disease (ECD) is a rare non-Langerhans histiocytosis with multisystem inflammatory infiltrates consistent of monocytes/macrophages, reactive microenvironment and fibrotic fields. Cardiovascular involvement is one of the most frequent manifestations of ECD that can lead to life threating complications. In this article we are reporting a clinical case of ECD with cardiac involvement in a young patient.
ISSN:0040-3660
2309-5342
DOI:10.26442/00403660.2022.07.201737