Plasma exchange as a source of protein C for acute onset protein C pathway failure.
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.
Hodgson
Andy
A
National Centre for Hereditary Coagulation Disorders, St James's Hospital, Dublin, Ireland.
Ryan
Tom
T
Moriarty
Jan
J
Mellotte
George
G
Murphy
Ciaran
C
Smith
Owen P
OP
D000208:Acute Disease; D000328:Adult; D005260:Female; D006801:Humans; D011695:IgA Vasculitis / Q000628:therapy; D009102:Multiple Organ Failure / Q000628:therapy; D010951:Plasma Exchange; D011008:Pneumococcal Infections / Q000628:therapy; D020151:Protein C Deficiency / Q000628:therapy*; D012772:Shock, Septic / Q000628:therapy