Hypercoagulable state (thrombophilia)

Guidelines

"How I Treat"

• 2020: May & Moll How I treat unexplained arterial thrombosis

Inherited thrombophilias

• Prothrombin gene mutation (G20210A)
• Factor V Leiden mutation
• Antithrombin III deficiency
• Protein C deficiency
• Protein S deficiency
• Dysfibrinogenemia

Controversial:

• Plasminogen deficiency
• Factor XII deficiency

Acquired thrombophilias and clotting risk factors

• Surgery, foreign bodies such as venous catheters, smoking, obesity, elevated factor VIII, advanced age, male sex, trauma, sedentary state, immobilization, malignancy, myeloproliferative disorders-- polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), pregnancy, medications--e.g. Bevacizumab (Avastin), Lenalidomide (Revlimid), Thalidomide (Thalomid), Tamoxifen (Nolvadex), oral contraceptive use, heparin--in the case of heparin-induced thrombocytopenia/thrombosis (HIT), congestive heart failure, anatomic/low cardiovascular flow states--e.g. inferior vena cava (IVC) abnormalities, chronic inflammation, inflammatory bowel disease, nephrotic syndrome, paroxysmal noctural hemoglobinuria (PNH)
• Prior venous thromboembolism (VTE)
• Antiphospholipid antibody syndrome (APS)
  • Lupus anticoagulant: dilute Russell viper venom time (dRVVT), dilute activated partial thromboplastin time (dAPTT), Staclot LA (hexagonal phase phospholipids), Kaolin clotting time (KCT)
  • Anticardiolipin (aCL) antibody IgG, IgM, IgA
  • Beta 2 glycoprotein 1 (B2GP1) IgG, IgM, IgA
  • Sapporo criteria for APS

Related pages

• Antiphospholipid antibody syndrome
• Inherited thrombophilia
• Venous thromboembolism

References

• Moll S. Thrombophilia: clinical-practical aspects. J Thromb Thrombolysis. 2015 Apr;39(3):367-78.
  • Table of reliability of thrombophilia testing with anticoagulants and in acute thrombosis