Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases

被引：42

作者：

Franchini, Massimo ^{[1
]}

Montagnana, Martina ^{[2
]}

Targher, Giovanni ^{[3
]}

Lippi, Giuseppe ^{[2
]}

机构：

[1] Azienda Osped Verona, Serv Immunoematol & Trasfus, Ctr Emofilia, I-37126 Verona, Italy

[2] Univ Verona, Sez Chim Clin, Dipartimento Sci Morfol Biomed, I-37100 Verona, Italy

[3] Univ Verona, Dipartimento Sci Biomed & Chirurg, Sez Endocrinol & Malattie Metab, I-37100 Verona, Italy

来源：

SEMINARS IN THROMBOSIS AND HEMOSTASIS | 2007年 / 33卷 / 08期

关键词：

ADAMTS-13; von Willebrand factor; thrombotic microangiopathy;

D O I：

10.1055/s-2007-1000365

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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) under bar (d) under bar isintegrin (a) under bar nd (m) under bar etalloproteinase with (t) under bar hrombo (s) under bar pondin-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.

引用

页码：787 / 797

页数：11

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