ADAMTS-13 assays in thrombotic thrombocytopenic purpura

F. Peyvandi, R. Palla, L. A. Lotta, I. Mackie, M. A. Scully, S. J. Machin

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)631-640
Number of pages10
JournalJournal of Thrombosis and Haemostasis
Volume8
Issue number4
DOIs
Publication statusPublished - Apr 2010

Keywords

  • ADAMTS-13
  • Assays
  • Clinical utility
  • Standardization

ASJC Scopus subject areas

  • Hematology

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