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Semin Thromb Hemost 2004; 30(5): 549-557
DOI: 10.1055/s-2004-835675
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Molecular Mechanisms in Thrombotic Thrombocytopenic Purpura

Han-Mou Tsai1
  • 1Associate Professor of Medicine, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York
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Publication History

Publication Date:
21 October 2004 (online)

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Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolysis. Unlike the typical hemolytic uremic syndrome (HUS), which follows infection with shiga toxin-producing microorganisms, most cases of TTP do not have an obvious etiology. Recent studies revealed that a plasma zinc metalloprotease ADAMTS (a disintegrin and metalloprotease with thrombospondin type 1 motif) 13 cleaves von Willebrand factor in a shear-dependent manner. Deficiency of ADAMTS13, due to autoimmune inhibitors of the protease or genetic mutation in the ADAMTS13 gene, results in a propensity to the development of von Willebrand factor-platelet aggregation and intravascular thrombosis characteristic of TTP. The identification of the molecular defect in TTP raises the prospect that this hitherto mysterious disorder will be managed with a more rationally designed strategy in the near future.

KEYWORDS

Thrombotic thrombocytopenic purpura - TTP - metalloprotease - ADAMTS - von Willebrand factor

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Han-Mou TsaiM.D. 

Montefiore Medical Center, Division of Hematology

111 East 210th Street

Bronx, NY 10467

Email: htsai@montefiore.org