ADAMTS-13 assays in thrombotic thrombocytopenic purpura

被引：85

作者：

Peyvandi, F. ^{[1
,2
]}

Palla, R. ^{[1
]}

Lotta, L. A. ^{[1
]}

Mackie, I. ^{[2
]}

Scully, M. A. ^{[2
]}

Machin, S. J. ^{[2
]}

机构：

[1] Univ Milan, Dept Med & Med Special,Luigi Villa Fdn, Angelo Bianchi Bonomi Hemophilia & Thrombosis Ctr, Fdn IRCCS Ca Granda,Osped Maggiore Policlin, Milan, Italy

[2] UCL, Dept Haematol, Haemostasis Res Unit, London, England

来源：

JOURNAL OF THROMBOSIS AND HAEMOSTASIS | 2010年 / 8卷 / 04期

关键词：

ADAMTS-13; assays; clinical utility; standardization; VON-WILLEBRAND-FACTOR; FACTOR-CLEAVING PROTEASE; HEMOLYTIC-UREMIC SYNDROME; ANTI-ADAMTS13; ANTIBODIES; FRETS-VWF73; ASSAY; ENZYME-IMMUNOASSAY; PROGNOSTIC VALUE; PLASMA-EXCHANGE; IGG ANTIBODIES; MICROANGIOPATHIES;

D O I：

10.1111/j.1538-7836.2010.03761.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but life threatening disease characterized by microangiopathic haemolytic anaemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis and variable signs and symptoms of organ ischemia and damage. In the last few years, a number of in house and commercial laboratory assays for ADAMTS-13 and its autoantibodies have been developed. The features and clinical utility of ADAMTS-13 assays are summarized in this review.

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页码：631 / 640

页数：10

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