HUS and the case for complement

Blood. 2015 Oct 29;126(18):2085-90. doi: 10.1182/blood-2015-03-569277. Epub 2015 Sep 22.

Abstract

Hemolytic-uremic syndrome (HUS) is a thrombotic microangiopathy that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Excess complement activation underlies atypical HUS and is evident in Shiga toxin-induced HUS (STEC-HUS). This Spotlight focuses on new knowledge of the role of Escherichia coli-derived toxins and polyphosphate in modulating complement and coagulation, and how they affect disease progression and response to treatment. Such new insights may impact on current and future choices of therapies for STEC-HUS.

MeSH terms

  • Blood Coagulation*
  • Complement Activation*
  • Complement System Proteins / genetics
  • Complement System Proteins / immunology*
  • Escherichia coli Infections / blood
  • Escherichia coli Infections / complications*
  • Escherichia coli Infections / genetics
  • Escherichia coli Infections / immunology
  • Hemolytic-Uremic Syndrome / blood
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / immunology*
  • Hemolytic-Uremic Syndrome / microbiology*
  • Humans
  • Mutation
  • Polyphosphates / immunology
  • Shiga-Toxigenic Escherichia coli / immunology*

Substances

  • Polyphosphates
  • Complement System Proteins