Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease
  • Maria Kyriakopoulou
    Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Guy Decaux
    Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Mike El Mourad
    Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Ruben Casado Arroyo
    Cardiology Department, Cliniques Universitaires Erasme, Bruxelles

Keywords

Erdheim Chester disease, tamponade, BRAF, interferon alpha

Abstract

Erdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosis and a very rare multisystemic disease of unknown aetiology, with skeletal involvement of the long bones and in more than 50% of cases with extraskeletal involvement. The disease was described in 1930 by the anatomopathologist Jakob Erdheim and his student William Chester. More than 500 cases have since been reported. We report the case of a 77-year-old Italian woman with ECD who was admitted to hospital for acute cardiac tamponade. The patient presented with simultaneous cutaneous, retro-orbital, skeletal, cerebral and cardiovascular manifestations and was successfully treated with corticosteroids followed by interferon.

VIEW THE ENTIRE ARTICLE

References

  • Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester disease: a comprehensive review of the literature. Orphanet J Rare Dis 2013;8:137.
  • Arnaud L, Hervier B, Neel A, Hamidou MA, Kahn JE, Wechsler B, et al. CNS involvement and treatment with interferon-alpha are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.Blood 2011;117:2778–2782.
  • Haroche J, Arnaud L, Amoura Z. Erdheim-Chester disease. Curr Opin Rheumatol 2012;24:53–59.
  • MunozJ, Janku F, Cohen P,Kurzrock R. Erdheim-Chester disease: characteristics and management. Mayo Clin Proc 2014;89985–996.
  • Haroche J, Cohen-Aubart F, Emile JF, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood 2013;121:1495–1500

    • Views: 1032
    HTML downloads: 283
    PDF downloads: 455
    Untitled downloads: 0
    Untitled downloads: 0
    Figure 2 downloads: 0
    Figure 1 downloads: 0

    Published: 2016-11-28
    Issue: Vol. 3 No. 7 (view)

    How to cite:
    1.
    Kyriakopoulou M, Decaux G, El Mourad M, Casado Arroyo R. Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease. EJCRIM 2016;3 doi:10.12890/2016_000451.