Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases.
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 Thrombo Spondin-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.
Franchini
Massimo
M
Servizio di Immunoematologia e Trasfusione - Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy. massimo.franchini@univr.it
Montagnana
Martina
M
Targher
Giovanni
G
Lippi
Giuseppe
G
D051722:ADAM Proteins / Q000097:blood* / Q000172:deficiency; D000071120:ADAMTS13 Protein; D001327:Autoimmune Diseases / Q000201:enzymology; D016026:Bone Marrow Transplantation; D005260:Female; D006801:Humans; D007239:Infections / Q000097:blood; D009369:Neoplasms / Q000201:enzymology; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000201:enzymology*; D013927:Thrombosis / Q000201:enzymology*; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human