Plasma exchange as a source of protein C for acute onset protein C pathway failure

Br J Haematol. 2002 Mar;116(4):905-8. doi: 10.1046/j.0007-1048.2002.03353.x.

Abstract

Severe bacterial sepsis, particularly secondary to meningococcaemia, is a well-recognized cause of purpura fulminans resulting from severe acquired protein C (PC) deficiency. Recently, PC and activated protein C (APC) concentrate replacement therapy has been shown to improve outcome in patients with meningococcaemia- associated purpura fulminans and severe sepsis respectively. Despite these impressive findings, PC and APC concentrates are not currently widely available. We describe a 31-year-old patient with pneumococcal septic shock, purpura fulminans (PF) and severe acquired PC deficiency, whom we successfully treated with conventional therapy and high-volume plasma exchange as a source of PC.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Adult
  • Female
  • Humans
  • IgA Vasculitis / therapy
  • Multiple Organ Failure / therapy
  • Plasma Exchange*
  • Pneumococcal Infections / therapy
  • Protein C Deficiency / therapy*
  • Shock, Septic / therapy