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Hereditary and Acquired Hypercoagulability  Follow-up

  • Author: Paul Schick, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
 
Updated: May 26, 2016
 

Deterrence/Prevention

If a patient is known to have a lupus anticoagulant or a thrombophilia, it is important to avoid oral contraceptives and hormone replacement therapy. Also, prophylactic anticoagulation should be considered in patients with additional risk for venous thrombosis, such as immobilization or surgery.

The risk of venous thrombosis is considerably greater in patients with two hereditary thrombophilias or with a thrombophilia and an acquired hypercoagulable disorder. Prophylactic anticoagulation should be considered in these circumstances.

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Prognosis

The risk of thrombosis in a person with hypercoagulability varies with the underlying condition (see Epidemiology). The prognosis is probably worse in patients with antithrombin III deficiency and lupus anticoagulants than in those without these factors.

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Contributor Information and Disclosures
Author

Paul Schick, MD Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Barbara P Schick, PhD Professor, Department of Medicine, Cardeza Foundation; Professor, Department of Biochemistry and Molecular Pharmacology, Professor, Department of Biochemistry and Molecular Biology, Jefferson Medical College of Thomas Jefferson University

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada, American Clinical and Climatological Association, International Society of Blood Transfusion

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,,Grifols<br/>Received honoraria from all the above companies for speaking and teaching.

Chief Editor

Srikanth Nagalla, MBBS, MS, FACP Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

Srikanth Nagalla, MBBS, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Nothing to disclose.

Additional Contributors

Pradyumna D Phatak, MBBS, MD Chair, Division of Hematology and Medical Oncology, Rochester General Hospital; Clinical Professor of Oncology, Roswell Park Cancer Institute

Pradyumna D Phatak, MBBS, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching.

References
  1. Rosendaal FR. Venous thrombosis: a multicausal disease. Lancet. 1999 Apr 3. 353(9159):1167-73. [Medline].

  2. Hillarp A, Dahlback B, Zoller B. Activated protein C resistance: from phenotype to genotype and clinical practice. Blood Rev. 1995 Dec. 9(4):201-12. [Medline].

  3. Wu KK, Thiagarajan P. Role of endothelium in thrombosis and hemostasis. Annu Rev Med. 1996. 47:315-31. [Medline].

  4. Colman-Brochu S. Deep vein thrombosis in pregnancy. MCN Am J Matern Child Nurs. 2004 May-Jun. 29(3):186-92. [Medline].

  5. Bates SM, Greer IA, Pabinger I, Sofaer S, Hirsh J. Venous thromboembolism, thrombophilia, antithrombotic therapy, and pregnancy: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008 Jun. 133(6 suppl):844S-886S. [Medline].

  6. Rand JH, Senzel L. Antiphospholipid antibodies and the antiphospholipid syndrome. Colman RW, Marder VJ, Clowes AW, et al, eds. Hemostasis and Thrombosis. 5th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins; 2006. 1621-36.

  7. Ortel TL. Thrombosis and the antiphospholipid syndrome. Hematology Am Soc Hematol Educ Program. 2005. 462-8. [Medline]. [Full Text].

  8. Triplett DA. Antiphospholipid antibodies. Clin Adv Hematol Oncol. 2003 Dec. 1(12):726-30. [Medline].

  9. Franchini M, Mannucci PM. ABO blood group and thrombotic vascular disease. Thromb Haemost. 2014 Dec. 112 (6):1103-9. [Medline].

  10. Heit JA, Cunningham JM, Petterson TM, et al. Genetic variation within the anticoagulant, procoagulant, fibrinolytic and innate immunity pathways as risk factors for venous thromboembolism. J Thromb Haemost. 2011 Jun. 9(6):1133-42. [Medline]. [Full Text].

  11. Eroglu A, Egin Y, Cam R, Akar N. The 19-bp deletion of dihydrofolate reductase (DHFR), methylenetetrahydrofolate reductase (MTHFR) C677T, Factor V Leiden, prothrombin G20210A polymorphisms in cancer patients with and without thrombosis. Ann Hematol. 2009 Jan. 88(1):73-6. [Medline].

  12. Mayo Clinic. Activated Protein C Resistance V (APCRV). Mayo Medical Laboratories. Available at http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/81967. Accessed: February 13, 2016.

  13. Heit JA. Thrombophilia: common questions on laboratory assessment and management. Hematology Am Soc Hematol Educ Program. 2007. 127-35. [Medline]. [Full Text].

  14. Couturaud F, Leroyer C, Julian JA, et al. Factors that predict risk of thrombosis in relatives of patients with unprovoked venous thromboembolism. Chest. 2009 Jul 10. epub ahead of print. [Medline].

  15. Lijfering WM, Veeger NJ, Middeldorp S, et al. A lower risk of recurrent venous thrombosis in women compared with men is explained by sex-specific risk factors at time of first venous thrombosis in thrombophilic families. Blood. 2009 Sep 3. 114(10):2031-6. [Medline]. [Full Text].

  16. Roberts LN, Patel RK, Arya R. Venous thromboembolism and ethnicity. Br J Haematol. 2009 Aug. 146(4):369-83. [Medline].

  17. Cushman M. Inherited risk factors for venous thrombosis. Hematology Am Soc Hematol Educ Program. 2005. 452-7. [Medline]. [Full Text].

  18. Aiach M, Emmerrich J. Thrombophilia genetics. Colman RW, Marder VJ, Clowes AW, et al, eds. Hemostasis and Thrombosis. 5th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins; 2006. 779-93.

  19. Straczek C, Oger E, Yon de Jonage-Canonico MB, et al. Prothrombotic mutations, hormone therapy, and venous thromboembolism among postmenopausal women: impact of the route of estrogen administration. Circulation. 2005 Nov 29. 112(22):3495-500. [Medline]. [Full Text].

  20. Urbanus RT, de Groot PG. Antiphospholipid antibodies--we are not quite there yet. Blood Rev. 2011 Mar. 25(2):97-106. [Medline].

  21. Bates SM. Management of pregnant women with thrombophilia or a history of venous thromboembolism. Hematology Am Soc Hematol Educ Program. 2007. 143-50. [Medline]. [Full Text].

  22. Douketis J, Tosetto A, Marcucci M, et al. Risk of recurrence after venous thromboembolism in men and women: patient level meta-analysis. BMJ. 2011 Feb 24. 342:d813. [Medline]. [Full Text].

  23. Kearon C, Julian JA, Kovacs MJ, et al. Influence of thrombophilia on risk of recurrent venous thromboembolism while on warfarin: results from a randomized trial. Blood. 2008 Dec 1. 112(12):4432-6. [Medline]. [Full Text].

  24. Hirsh J, Raschke R. Heparin and low-molecular-weight heparin: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy. Chest. 2004 Sep. 126(3 suppl):188S-203S. [Medline]. [Full Text].

  25. Warkentin TE. Agents for the treatment of heparin-induced thrombocytopenia. Hematol Oncol Clin North Am. 2010 Aug. 24(4):755-75, ix. [Medline].

  26. Adam SS, McDuffie JR, Lachiewicz PF, Ortel TL, Williams JW. Comparative Effectiveness of Newer Oral Anticoagulants and Standard Anticoagulant Regimens for Thromboprophylaxis in Patients Undergoing Total Hip or Knee Replacement [Internet]. VA Evidence-based Synthesis Program Reports. 2012 Dec. [Medline].

  27. Perez A, Merli GJ. Novel Anticoagulant Use for Venous Thromboembolism: A 2013 Update. Curr Treat Options Cardiovasc Med. 2013 Feb 6. [Medline].

  28. Dobesh PP, Oestreich JH. Novel Direct-Acting Anticoagulants for Risk Reduction in ACS. J Pharm Pract. 2012 Nov 19. [Medline].

  29. Merli GJ. The new oral anticoagulants: a challenge for hospital formularies. Hosp Pract (Minneap). 2012 Aug. 40(3):126-8. [Medline].

  30. Komócsi A, Vorobcsuk A, Kehl D, Aradi D. Use of new-generation oral anticoagulant agents in patients receiving antiplatelet therapy after an acute coronary syndrome: systematic review and meta-analysis of randomized controlled trials. Arch Intern Med. 2012 Nov 12. 172(20):1537-45. [Medline].

  31. Kar S, Bhatt DL. Anticoagulants for the treatment of acute coronary syndrome in the era of new oral agents. Coron Artery Dis. 2012 Sep. 23(6):380-90. [Medline].

  32. Rajasekhar A, Beyth R, Crowther MA. Newer anticoagulants in critically ill patients. Crit Care Clin. 2012 Jul. 28(3):427-51, vii. [Medline].

  33. Lee AY. Treatment of established thrombotic events in patients with cancer. Thromb Res. 2012 Apr. 129 Suppl 1:S146-53. [Medline].

  34. Cohen AT, Gurwith MM, Dobromirski M. Thromboprophylaxis in non-surgical cancer patients. Thromb Res. 2012 Apr. 129 Suppl 1:S137-45. [Medline].

  35. Vande Griend JP, Marcum ZA, Linnebur SA. A year in review: new drugs for older adults in 2011. Am J Geriatr Pharmacother. 2012 Aug. 10(4):258-63. [Medline].

  36. Burke DA, Warraich HJ, Pinto DS. Which antithrombin for whom? Identifying the patient population that benefits most from novel antithrombin agents. Curr Cardiol Rep. 2012 Aug. 14(4):493-501. [Medline].

  37. Di Nisio M, Middeldorp S, Büller HR. Direct thrombin inhibitors. N Engl J Med. 2005 Sep 8. 353 (10):1028-40. [Medline].

  38. Baglin T, Barrowcliffe TW, Cohen A, Greaves M. Guidelines on the use and monitoring of heparin. Br J Haematol. 2006 Apr. 133(1):19-34. [Medline]. [Full Text].

  39. Harenberg J. Is laboratory monitoring of low-molecular-weight heparin therapy necessary? Yes. J Thromb Haemost. 2004 Apr. 2(4):547-50. [Medline]. [Full Text].

  40. Bounameaux H, de Moerloose P. Is laboratory monitoring of low-molecular-weight heparin therapy necessary? No. J Thromb Haemost. 2004 Apr. 2(4):551-4. [Medline]. [Full Text].

  41. [Guideline] Kearon C, Akl EA, Comerota AJ, Prandoni P, Bounameaux H, Goldhaber SZ, et al. Antithrombotic therapy for VTE disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb. 141 (2 Suppl):e419S-94S. [Medline]. [Full Text].

  42. [Guideline] Kitchen S, Gray E, Mackie I, Baglin T, Makris M, BCSH committee. Measurement of non-coumarin anticoagulants and their effects on tests of Haemostasis: Guidance from the British Committee for Standards in Haematology. Br J Haematol. 2014 Sep. 166 (6):830-41. [Medline]. [Full Text].

  43. Cho L, Kottke-Marchant K, Lincoff AM, et al. Correlation of point-of-care ecarin clotting time versus activated clotting time with bivalirudin concentrations. Am J Cardiol. 2003 May 1. 91(9):1110-3. [Medline].

  44. Welsby IJ, McDonnell E, El-Moalem H, Stafford-Smith M, Toffaletti JG. Activated clotting time systems vary in precision and bias and are not interchangeable when following heparin management protocols during cardiopulmonary bypass. J Clin Monit Comput. 2002 Jul. 17(5):287-92. [Medline].

  45. Chang LC, Lee HF, Yang Z, Yang VC. Low molecular weight protamine (LMWP) as nontoxic heparin/low molecular weight heparin antidote (I): preparation and characterization. AAPS PharmSci. 2001. 3(3):E17. [Medline].

  46. Schick BP, Maslow D, Moshinski A, San Antonio JD. Novel concatameric heparin-binding peptides reverse heparin and low-molecular-weight heparin anticoagulant activities in patient plasma in vitro and in rats in vivo. Blood. 2004 Feb 15. 103(4):1356-63. [Medline]. [Full Text].

  47. Welsby IJ, Stafford-Smith M. Monitoring direct thrombin inhibitors: time for standardization. Anesthesiology. 2004 Oct. 101(4):1048-9. [Medline].

  48. Weitz JI. New anticoagulants for treatment of venous thromboembolism. Circulation. 2004 Aug 31. 110(9 suppl 1):I19-26. [Medline]. [Full Text].

  49. Connors JM. Antidote for Factor Xa Anticoagulants. N Engl J Med. 2015 Dec 17. 373 (25):2471-2. [Medline]. [Full Text].

  50. Lassen MR, Raskob GE, Gallus A, Pineo G, Chen D, Hornick P. Apixaban versus enoxaparin for thromboprophylaxis after knee replacement (ADVANCE-2): a randomised double-blind trial. Lancet. 2010 Mar 6. 375(9717):807-15. [Medline].

  51. Romualdi E, Ageno W. Oral Xa inhibitors. Hematol Oncol Clin North Am. 2010 Aug. 24(4):727-37, viii-ix. [Medline].

  52. Medi C, Hankey GJ, Freedman SB. Stroke risk and antithrombotic strategies in atrial fibrillation. Stroke. 2010 Nov. 41(11):2705-13. [Medline].

  53. Becattini C, Lignani A, Agnelli G. New anticoagulants for the prevention of venous thromboembolism. Drug Des Devel Ther. 2010. 4:49-60. [Medline].

  54. Desai SS, Massad MG, DiDomenico RJ, et al. Recent developments in antithrombotic therapy: will sodium warfarin be a drug of the past?. Recent Pat Cardiovasc Drug Discov. 2006 Nov. 1(3):307-16. [Medline].

  55. Hanley JP. Warfarin reversal. J Clin Pathol. 2004 Nov. 57(11):1132-9. [Medline]. [Full Text].

  56. Keeney M, Allan DS, Lohmann RC, Yee IH. Effect of activated recombinant human factor 7 (Niastase) on laboratory testing of inhibitors of factors VIII and IX. Lab Hematol. 2005. 11(2):118-23. [Medline].

  57. Malherbe S, Tsui BC, Stobart K, Koller J. Argatroban as anticoagulant in cardiopulmonary bypass in an infant and attempted reversal with recombinant activated factor VII. Anesthesiology. 2004 Feb. 100(2):443-5. [Medline].

  58. Powner DJ, Hartwell EA, Hoots WK. Counteracting the effects of anticoagulants and antiplatelet agents during neurosurgical emergencies. Neurosurgery. 2005 Nov. 57(5):823-31; discussion 823-31. [Medline].

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Table 1.  Prevalence of Acquired or Hereditary Hypercoagulable Disorders and Risks of Venous Thrombosis.
ConditionPrevalence in General Population (%)Relative Risk of VTE (%)Relative Risk of Recurrent VTE (%)
Factor V Leiden



(heterozygous)



3-74.31.3
Prothrombin 20210A



(heterozygous)



1-31.91.4
Protein C deficiency



(heterozygous)



0.02-0.0511.32.5
Protein S deficiency



(heterozygous)



0.01-132.42.5
Antithrombin deficiency



(heterozygous)



0.02-0.0417.52.5
VTE = Venous thromboembolism
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