Reduced von Willebrand Factor-Cleaving Protease Levels in Secondary Thrombotic Microangiopathies and Other Diseases

 

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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.

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Massimo FranchiniM.D. 

Servizio di Immunoematologia e Trasfusione - Centro Emofilia, Azienda Ospedaliera di Verona, P.le Stefani 1

37126 - Verona, Italy

Email: massimo.franchini@univr.it