Effective treatment of hereditary angioedema with fresh frozen plasma in an emergency department

J Emerg Med. 2007 Aug;33(2):137-9. doi: 10.1016/j.jemermed.2007.02.024. Epub 2007 May 30.

Abstract

Hereditary angioedema (HAE) is a rarely seen disorder of C1 inhibitor (C1-INH) deficiency usually manifested by non-pruritic swelling of the skin. Acute exacerbations are not sensitive to conventional medications, and C1-esterase inhibitor concentrates are recommended as the first-line therapy. However, fresh frozen plasma is the main treatment alternative in many centers due to the lack of C1-esterase inhibitor concentrates. In this report, we present 3 patients with acute exacerbations of hereditary angioedema who were effectively and safely treated with fresh frozen plasma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Angioedema / complications
  • Angioedema / therapy*
  • Complement C1 Inactivator Proteins / deficiency
  • Complement C1 Inhibitor Protein
  • Emergency Service, Hospital
  • Female
  • Genetic Diseases, Inborn / therapy
  • Humans
  • Plasma*
  • Serpins / deficiency
  • Treatment Outcome

Substances

  • Complement C1 Inactivator Proteins
  • Complement C1 Inhibitor Protein
  • SERPING1 protein, human
  • Serpins