Semin Thromb Hemost 2005; 31(6): 652-658
DOI: 10.1055/s-2005-925471
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Thrombophilia and Thrombosis in Thrombotic Thrombocytopenic Purpura

Anil Potti1 , Veshana Ramiah1 , Thomas L. Ortel1 , 2
  • 1Division of Hematology, Department of Medicine, Duke University Medical Center, Durham, North Carolina
  • 2Associate Professor, Department of Medicine, Duke University Medical Center, Durham, North Carolina
Further Information

Publication History

Publication Date:
30 December 2005 (online)

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia often accompanied by microvascular ischemia, which may manifest as sensorimotor signs, visual changes, renal impairment, cardiac ischemia, and abdominal pain, depending on the organs affected. Until a few decades ago, TTP remained an almost universally fatal disorder. The introduction of plasma exchange therapy (PE) with replacement of fresh frozen plasma has improved the survival of patients with acute TTP dramatically from less than 10% to approximately 80 to 90% and is now considered the therapy of choice. During the last decade, the understanding of the pathophysiology of thrombotic microangiopathies, especially TTP, has increased considerably. The clinical features of thrombotic sequelae in TTP are diverse and usually secondary to microvascular thrombosis. Clinical and laboratory evidence for disruption of primary and secondary hemostasis, the role of endothelial integrity and fibrinolysis, the relevance of large vessel thrombosis, and the prevalence of thrombophilic states in TTP are discussed in this review. In summary, although the syndrome of TTP can sometimes be confused with other thrombotic diatheses, it is clear that the phenomenon of thrombosis in TTP appears to be distinctly different, both biologically and clinically, from other causes of microangiopathy and/or antibody-mediated thrombosis. Traditional anticoagulant and antiplatelet strategies are generally not effective in patients with TTP, despite the predominance of thrombotic manifestations, and common prothrombotic polymorphisms detected in patients with venous thromboembolism are seldom present.

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Thomas OrtelM.D. Ph.D. 

Associate Professor, Hemostasis & Thrombosis Center, Duke University Health System, Box 3422, Room 0563 Stead Building, Durham, NC 27710

Email: thomas.ortel@duke.edu

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