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Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

Ajay Kumar Mishra1, Sunithi Mani2, Anu Anna George3

  • 1Department of Internal Medicine, Christian Medical College, Vellore, Tamil Nadu, India.

BMJ Case Reports
|November 26, 2015
PubMed

Insights

Erdheim-Chester disease (ECD), a rare disorder, can affect the heart. This case highlights ECD presenting as recurrent pericardial effusion and tamponade, a critical cardiac manifestation.

Area of Science:

  • Cardiovascular pathology
  • Rare diseases
  • Histiocytic disorders

Background:

  • Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disorder characterized by lipid-laden histiocyte proliferation.
  • While bone pain is common, cardiac involvement like periaortic fibrosis and pericardial issues can occur in a subset of patients.

Observation:

  • This report details a specific case of Erdheim-Chester disease.
  • The patient presented with recurrent pericardial effusion.

Findings:

  • The case demonstrated significant pericardial effusion leading to cardiac tamponade.
  • This highlights a less common but severe cardiac manifestation of ECD.

Implications:

  • Recognizing cardiac involvement in ECD is crucial for timely diagnosis and management.
  • Pericardial effusion and tamponade should be considered in the differential diagnosis of unexplained cardiac issues in ECD patients.

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