Sepsis-induced disseminated intravascular coagulation with features of thrombotic thrombocytopenic purpura: a fatal fulminant syndrome

Clin Appl Thromb Hemost. 2011 Jun;17(3):251-3. doi: 10.1177/1076029609357739. Epub 2010 Mar 8.

Abstract

Disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP) are different disease states, while ADAMTS13 deficiency could occur in sepsis-induced DIC. We report 2 patients who had septic DIC with features of idiopathic TTP characterized by low ADAMTS13 activity and positive ADAMTS13 inhibitor. They had a specific fulminant course and fatal outcome, which might represent a new specific syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ADAM Proteins / blood
  • ADAMTS13 Protein
  • Adult
  • Disseminated Intravascular Coagulation / blood
  • Disseminated Intravascular Coagulation / etiology*
  • Fatal Outcome
  • Female
  • Humans
  • Middle Aged
  • Protease Inhibitors / blood
  • Purpura, Thrombotic Thrombocytopenic / blood
  • Purpura, Thrombotic Thrombocytopenic / etiology*
  • Sepsis / blood
  • Sepsis / complications*
  • Syndrome

Substances

  • Protease Inhibitors
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human