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Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series

Isabela Bispo Santos da Silva Costa1, André Neder Ramires Abdo1, Cristina Salvadori Bittar1

  • 1Instituto do Câncer do Estado de São Paulo, São Paulo, SP - Brazil.

Arquivos Brasileiros De Cardiologia
|December 6, 2018
PubMed

Insights

Erdheim-Chester Disease is a rare inflammatory myeloid neoplasm affecting men over 50. It commonly presents with skeletal, genitourinary, and central nervous system issues, alongside cardiovascular manifestations.

Area of Science:

  • Oncology
  • Pathology
  • Hematology

Background:

  • Erdheim-Chester Disease (ECD) is a rare inflammatory myeloid neoplasm.
  • Its incidence remains unknown, typically affecting men over 50.

Observation:

  • ECD is a multisystemic disorder.
  • The skeletal system is most frequently involved (90%), followed by genitourinary (60%) and central nervous system (25%) manifestations.
  • Cardiovascular involvement occurs in over half of patients.

Findings:

  • Skeletal involvement is the most common presentation of ECD.
  • Genitourinary and central nervous system (pituitary, diabetes insipidus) involvement are also significant.
  • Aortic infiltration and atrial pseudotumor are the predominant cardiovascular manifestations.

Implications:

  • Understanding ECD's multisystemic nature is crucial for diagnosis and management.
  • Further research into ECD's unknown incidence and pathogenesis is warranted.
  • Early recognition of cardiovascular manifestations can improve patient outcomes.

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