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Hypercoagulable states

Last updated: November 3, 2020

Etiology

The etiology of thrombophilia can be classified into two categories:

References:[1]

Clinical features

Thrombophilia is characterized by recurrent thromboembolism.

References:[2][3]

Hereditary thrombophilia

Defect Pathophysiology Prevalence in general population

Activated protein C resistance (APC-R)

Factor V Leiden

Heterozygosity: ∼ 5%
Homozygosity: < 1%
Elevated factor VIII ∼ 5%
Prothrombin mutation ∼ 3%
Protein S deficiency ∼ 1%
Protein C deficiency

< 1%

Antithrombin III deficiency ∼ 0.1%

Hyperhomocysteinemia

∼ 5–7%

References:[4][5][6][7][8]

Acquired thrombophilia

Etiology Pathophysiology
Surgery
  • Extended immobilization during procedure → blood stasis
  • Vessel instrumentation → endothelial damage
Trauma
  • Results in decreased venous blood flow, immobilization (blood stasis), and release of tissue factor (hypercoagulability) → increased clotting

Malignancy

Immobilization

  • Prolonged immobilization (e.g., extended travel, hospitalization, bed rest) → increased venous stasis
Smoking
Obesity

Antiphospholipid syndrome

Nephrotic syndrome

Oral contraceptive pills (OCPs) or hormone replacement therapy (HRT)

Heparin-induced thrombophilia
Pregnancy
Advanced age
  • Progressive endothelial damage
  • Increase in pro-clotting factors without a concomitant increase in protein C
  • Increase in other pro-clotting comorbidities (e.g., malignancy)
  • Decreased physical activity

References:[3][9][10][11]

Diagnostics

References:[2]

Treatment

Approach

Special considerations

The risk of venous thromboembolism is reduced with administration of an anticoagulant (e.g., heparin) following surgery!

References:[1][12][13]

Prevention

Consider prophylaxis in the following high-risk groups:

  • Postoperative patients
  • Prolonged immobilization or hospitalization
  • Malignancy
  • Orthopedic conditions

References

  1. Rosenson RS, Kang DS. Overview of Homocysteine. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/overview-of-homocysteine.Last updated: October 12, 2016. Accessed: April 6, 2017.
  2. Colman RW. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. Lippincott Williams & Wilkins ; 2006
  3. Den Boer NC, Van der Heiden C, Leijnse B, Souverijn JHM. Clinical chemistry: an overview . Plenum Press ; 1989
  4. Bauer KA, Leung LLK, Mahoney DH Jr, Tirnauer JS. Protein S Deficiency. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/protein-s-deficiency?.Last updated: November 16, 2016. Accessed: September 14, 2017.
  5. Kumar A, Afreen S, Mohd Yusuf, Gupta A. Mechanism and pathophysiology of activated protein C-related factor V leiden in venous thrombosis. Asian Journal of Transfusion Science. 2012; 6 (1): p.47. doi: 10.4103/0973-6247.95053 . | Open in Read by QxMD
  6. Schick P. Hereditary and Acquired Hypercoagulability. Hereditary and Acquired Hypercoagulability. New York, NY: WebMD. http://emedicine.medscape.com/article/211039-overview. Updated: May 26, 2016. Accessed: April 6, 2017.
  7. Siskin GP. Inferior Vena Cava Filters. Inferior Vena Cava Filters. New York, NY: WebMD. http://emedicine.medscape.com/article/419796-overview. Updated: December 3, 2015. Accessed: April 7, 2017.
  8. Kim JY, Khavanin N, Rambachan A, et al. Surgical duration and risk of venous thromboembolism. JAMA Surg. 2015; 150 (2): p.110-117. doi: 10.1001/jamasurg.2014.1841 . | Open in Read by QxMD
  9. Bauer KA, Lip GY. Evaluating patients with established venous thromboembolism for acquired and inherited risk factors. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/evaluating-patients-with-established-venous-thromboembolism-for-acquired-and-inherited-risk-factors.Last updated: September 20, 2016. Accessed: April 6, 2017.
  10. Bauer KA, Lip GY. Overview of the causes of venous thrombosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/overview-of-the-causes-of-venous-thrombosis.Last updated: September 20, 2016. Accessed: April 6, 2017.
  11. Blokhin IO, Lentz SR. Mechanisms of thrombosis in obesity. Curr Opin Hematol. 2015; 20 (5): p.437-444. doi: 10.1097/MOH.0b013e3283634443 . | Open in Read by QxMD
  12. Esmon CT. Basic Mechanisms and Pathogenesis of Venous Thrombosis. Blood Rev. 2009; 23 (5): p.225–229. doi: 10.1016/j.blre.2009.07.002 . | Open in Read by QxMD
  13. Tchaikovski SN, Rosing J. Mechanisms of estrogen-induced venous thromboembolism. Thromb Res. 2010; 126 (1): p.5-11. doi: 10.1016/j.thromres.2010.01.045 . | Open in Read by QxMD