Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem thrombotic microangiopathy (TMA). Significant advances have been made in understanding the pathogenesis of TTP since the discovery of ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin-1-like domains), the enzyme that regulates the size of von Willebrand factor (VWF) multimers. The inherited forms of TTP are mainly caused by a severe ADAMTS-13 deficiency, yet many aspects of the complex biological relationships between VWF-cleaving metalloproteinase and acquired TMA are still unclear. This latter issue will be critically addressed in this review article. In addition, the published literature evaluating plasma ADAMTS-13 levels in other pathologic conditions different from TMA will also be discussed. Copyright © 2007 by Thieme Medical Publishers, Inc.

Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases. [Review]

Montagnana M.;Targher G.;Lippi G.
2007-01-01

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem thrombotic microangiopathy (TMA). Significant advances have been made in understanding the pathogenesis of TTP since the discovery of ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin-1-like domains), the enzyme that regulates the size of von Willebrand factor (VWF) multimers. The inherited forms of TTP are mainly caused by a severe ADAMTS-13 deficiency, yet many aspects of the complex biological relationships between VWF-cleaving metalloproteinase and acquired TMA are still unclear. This latter issue will be critically addressed in this review article. In addition, the published literature evaluating plasma ADAMTS-13 levels in other pathologic conditions different from TMA will also be discussed. Copyright © 2007 by Thieme Medical Publishers, Inc.
2007
ADAMTS-13; Thrombotic microangiopathy; Von Willebrand factor;
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/313685
Citazioni
  • ???jsp.display-item.citation.pmc??? 6
  • Scopus 44
  • ???jsp.display-item.citation.isi??? 38
social impact