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Congenital Thrombophilic States Associated with Venous Thrombosis: A Qualitative Overview and Proposed Classification System

Mark A. Crowther, MD; and John G. Kelton, MD
[+] Article and Author Information

From St. Joseph's Hospital and McMaster University Medical Centre, Hamilton, Ontario, Canada.


Grant Support: Some of the studies cited in this report were funded by research grants from the Heart and Stroke Foundation of Ontario. Dr. Crowther holds a Canadian Institute of Health Research Scholarship. Dr. Kelton holds a Canada Research Chair.

Requests for Single Reprints: Mark A Crowther, MD, St. Joseph's Hospital, Room L208, 50 Charlton Avenue East, Hamilton, Ontario L8N 4A6, Canada.

Current Author Addresses: Dr. Crowther: St. Joseph's Hospital, Room L208, 50 Charlton Avenue East, Hamilton, Ontario L8N 4A6, Canada.

Dr. Kelton: McMaster University Medical Centre, Room 3W10, 1200 Main Street West, Hamilton, Ontario L8N 3Z5, Canada.


Ann Intern Med. 2003;138(2):128-134. doi:10.7326/0003-4819-138-2-200301210-00014
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Congenital causes of venous thrombosis have gained increasing prominence with the description of the factor V Leiden mutation and the prothrombin gene mutation. More recently, the description of the association between increased levels of coagulation factors and venous thrombosis and the finding that patients with thrombophilia can harbor more than one prothrombotic state have further increased the clinical relevance of the congenital thrombophilic states. In this qualitative review, we summarize current knowledge of the congenital prothrombotic states and propose a simple classification system that divides the states into two broad groups: those associated with reduced levels of the inhibitors of the coagulation cascade and those associated with increased levels or function of the coagulation factors. The first group is less common than the second, but it is associated with a much higher risk for venous thrombosis. This review provides clinicians with an evidence-based, practical guide to the congenital prothrombotic states.

Figures

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Figure 1.
The coagulation cascade.TF

Factor VIIa binds to tissue factor ( ) at sites of vascular injury, causing a cascade of reactions that ultimately leads to factor IIa (thrombin) generation. Thrombin activates platelets; converts fibrinogen to fibrin; leads to generation of additional thrombin through an autocatalytic loop; converts factor XIII to factor XIIIa, which stabilizes the thrombus; inhibits fibrinolysis; and acts as an anticoagulant by activating protein C. The principal inhibitors of coagulation are activated protein C (which, in concert with protein S, inactivates factors Va and VIIIa), antithrombin (which forms an inactive covalent complex with thrombin and factors Xa, IXa, and XIa), and tissue factor pathway inhibitor (which binds with and inactivates the tissue factor–factor VIIa complex).

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Figure 2.
Thrombosis-free survival for patients with the major thrombophilic states.

Point estimates of thrombosis-free survival are estimated and are based on the results presented by Martinelli and colleagues (5), Bucciarelli and colleagues (6), and Rodegheiro and Tosetto (7).

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Summary for Patients

Congenital Causes of Venous Thrombosis—A Classification System

The summary below is from the full report titled “Congenital Thrombophilic States Associated with Venous Thrombosis: A Qualitative Overview and Proposed Classification System.” It is in the 21 January 2003 issue of Annals of Internal Medicine (volume 138, pages 128-134). The authors are MA Crowther and JG Kelton.

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