Methodologies and Clinical Utility of ADAMTS-13 Activity Testing

Sarah Just

doi:10.1055/s-0030-1248727

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2010; 36(1): 082-090
DOI: 10.1055/s-0030-1248727

Methodologies and Clinical Utility of ADAMTS-13 Activity Testing

Sarah Just¹

¹Coagulation and Special Investigations, Pathology Queensland – Central Laboratory, Royal Brisbane and Women's Hospital Campus, Herston, Queensland, Australia

Abstract

ABSTRACT

Von Willebrand factor cleaving protease was first identified in 1987 and was further classified several years later as ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin-1-like domains). Congenital and acquired deficiency of ADAMTS-13 is associated with thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMAs). Assays for measurement of ADAMTS-13 were developed in the late 1990s, and significant improvements have occurred in the testing protocols to allow them to be performed in routine hemostasis laboratories. This article reviews the original ADAMTS-13 activity assays and those currently available. It also reviews the consistency of results among various methods and discusses the clinical utility of ADAMTS-13 testing in TTP, TMA, and other disease conditions.

KEYWORDS

ADAMTS-13 assays - thrombotic thrombocytopenic purpura - thrombotic microangiopathy

Full Text

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Ms. Sarah JustB. App. Sc. M.L.S.

Coagulation and Special Investigations, Pathology Queensland – Central Laboratory

Level 3, Block 7, Royal Brisbane and Women’s Hospital Campus, Butterfield St, Herston, Queensland Australia 4029

Email: Sarah_Just@health.qld.gov.au