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Signal Acquisition, Score Interpretation, and Economics of a Non-Invasive Point-of-Care Test for Coronary Artery Disease
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Successful Treatment of Erdheim-Chester Disease With Coronary Artery Involvement

Ai-Lin Zhao1, Yi-Ning Wang2, Feng-Dan Wang2

  • 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

The Canadian Journal of Cardiology
|December 12, 2018
PubMed

Insights

Erdheim-Chester disease (ECD), a rare inflammatory neoplasm, can affect the heart. Coronary artery involvement necessitates evaluation via CT angiography and treatment with interferon-alfa.

Area of Science:

  • Cardiovascular Medicine
  • Oncology
  • Radiology

Background:

  • Erdheim-Chester disease (ECD) is a rare inflammatory myeloid neoplasm with significant morbidity.
  • While bone involvement is common, extraskeletal manifestations, including cardiovascular complications, are increasingly recognized.

Observation:

  • A rare case of Erdheim-Chester disease presented with angina due to coronary artery involvement.
  • Radiologic and histologic findings confirmed ECD with a soft-tissue mass encasing the aorta and coronary arteries.

Findings:

  • Coronary artery involvement in ECD, though rare, can lead to significant cardiovascular symptoms.
  • Computed tomography angiography is effective for diagnosing cardiac manifestations of ECD.

Implications:

  • Early cardiovascular evaluation using coronary CT angiography is recommended for ECD patients.
  • Interferon-alfa demonstrates efficacy in treating Erdheim-Chester disease, including its cardiovascular manifestations.

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