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Blood, 15 September 2007, Vol. 110, No. 6, pp. 1723-1729.
Prepublished online as a Blood First Edition Paper on May 11, 2007; DOI 10.1182/blood-2006-10-053736.
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Submitted October 24, 2006
Accepted May 8, 2007
Trousseau's Syndrome: multiple definitions and multiple mechanisms
Ajit Varki*
Department of Medicine, University of California, San Diego, La Jolla, CA, United States
* Corresponding author; email: a1varki{at}ucsd.edu.
In 1865 Armand Trousseau noted that unexpected and/or migratory thrombophlebitis could be a forewarning of an occult visceral malignancy. An analysis by Sack and colleagues in 1977 extended the term "Trousseau's Syndrome" to include chronic disseminated intravascular coagulopathy associated with microangiopathy, verrucous endocarditis and arterial emboli in patients with cancer, often occurring with mucin-positive carcinomas. In recent times the term has been ascribed to clinical situations ranging all the way from these classic descriptions, to any kind of coagulopathy occurring in the setting of any kind of malignancy. These multiple definitions of "Trousseau's Syndrome" are partly the consequence of multiple pathophysiological mechanisms that apparently contribute to the hypercoagulability associated with cancer. Even the classic syndrome appears likely to represent a spectrum of disorders, ranging from exaggerated fluid-phased thrombosis dependent on prothrombotic agents such as tissue factor to a platelet- and endothelium-based selectin-dependent microangiopathy associated with mucin-producing carcinomas, along with thrombin and fibrin production. Also considered here are recent hypotheses concerning genetic pathways within tumor cells that might trigger these thrombotic phenomena, and the reasons why therapy with heparins of various kinds remain the preferred treatment - likely because of their salutary actions on several of the proposed pathological mechanisms.

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