Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis and inflammatory
myeloid neoplasm with poor prognosis. Symmetric long bone osteosclerosis occurs in
nearly all patients, but other organs are often involved. Coronary artery involvement
is rare, but was encountered in a patient who experienced angina. Radiologic presentation
and histologic findings were consistent with diagnosis of ECD. A soft-tissue mass
was found surrounding the right atrium, ascending aorta, and all branches of coronary
artery. Interferon-alfa treatment was successful. In conclusion, we recommend coronary
artery computed tomography angiography for cardiovascular evaluation of ECD and interferon-alfa
to treat ECD.
Résumé
La maladie d’Erdheim-Chester (MEC) est une histiocytose non langerhansienne rare et
une néoplasie myéloïde inflammatoire ayant un pronostic défavorable. Une ostéosclérose
symétrique des os longs survient chez presque tous les patients, mais d’autres organes
sont souvent atteints. L’atteinte des artères coronaires est rare, mais a été observée
chez un patient souffrant d’angine. Le tableau radiologique et les observations histologiques
correspondaient à un diagnostic de MEC. Une masse de tissu mou entourant l’oreillette
droite, l’aorte ascendante et toutes les branches des artères coronaires a été observée.
Le traitement par l’interféron alpha a été un succès. En conclusion, nous recommandons
une angiographie des artères coronaires par tomodensitométrie pour l’évaluation des
manifestations cardiovasculaires de la MEC et le recours à l’interféron alpha pour
traiter cette maladie.
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References
- Evaluation of clinicopathologic characteristics and the BRAF V600E mutation in Erdheim-Chester disease among Chinese adults.Ann Hematol. 2016; 95: 745-750
- Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease.Blood. 2014; 124: 483-492
- Erdheim-Chester disease: the importance of information integration.Case Rep Oncol. 2017; 10: 613-619
- Erdheim-Chester disease with cardiovascular involvement and BRAF V600E mutation.Circ J. 2016; 80: 1657-1659
Article info
Publication history
Published online: September 28, 2018
Accepted:
September 25,
2018
Received:
August 28,
2018
Footnotes
See page 1688.e11 for disclosure information.
Identification
Copyright
© 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
