Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis

Haematologica. 2007 Jan;92(1):121-4. doi: 10.3324/haematol.10262.


We studied the state of ultra-large von Willebrand factor (ULVWF) proteolysis in 21 pediatric patients with severe sepsis and found that the overall group of patients had moderately reduced ADAMTS-13 activity, but 31% had severe enzymatic deficiency. The severe deficiency correlated with greater adhesion activity of von Willebrand factor, severity of thrombocytopenia and plasma levels of interleukin-6. It also correlated clinically with severity of illness and organ dysfunction. These results suggest that ULVWF proteolysis is insufficient in septic patients and severely deficient in a subgroup of patients. The deficiency may contribute to the development of thrombocytopenia and ischemic organ failure associated with sepsis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ADAM Proteins / biosynthesis
  • ADAM Proteins / deficiency*
  • ADAMTS13 Protein
  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Gene Expression Regulation*
  • Humans
  • Interleukin-6 / blood
  • Male
  • Sepsis / blood*
  • Thrombocytopenia / blood
  • Thrombocytopenia / etiology
  • von Willebrand Factor / biosynthesis


  • Interleukin-6
  • von Willebrand Factor
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human