Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency

Hematol Oncol Clin North Am. 2007 Aug;21(4):609-32, v. doi: 10.1016/j.hoc.2007.06.003.


A serious disorder with characteristic microvascular thrombosis involving the brain and other organs, thrombotic thrombocytopenic purpura (TTP) typically presents with thrombocytopenia, hemolysis with schistocytes on blood smears, and mental changes or seizures. It may progress rapidly to a fatal end if the patient is not treated immediately with plasma. Recent advances have shown that TTP is caused by deficiency of a circulating, von Willebrand factor cleaving metalloprotease, ADAMTS13. This new knowledge will provide clues to improve the diagnosis and management of this intriguing disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • ADAM Proteins / deficiency*
  • ADAM Proteins / genetics
  • ADAM Proteins / immunology
  • ADAMTS13 Protein
  • Autoantibodies / blood
  • Disease Management
  • Humans
  • Purpura, Thrombotic Thrombocytopenic / diagnosis
  • Purpura, Thrombotic Thrombocytopenic / etiology*
  • Purpura, Thrombotic Thrombocytopenic / genetics
  • Thrombosis / etiology
  • von Willebrand Factor / chemistry


  • Autoantibodies
  • von Willebrand Factor
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human