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Overview of management for patients with inherited thrombophilia

Overview of management for patients with inherited thrombophilia
This algorithm applies to individuals with known inherited thrombophilia (factor V Leiden or prothrombin G20210A; protein S, protein C, or AT deficiency). It is a general overview of our approach and does not substitute for clinical judgment regarding the risks and benefits of anticoagulation for an individual patient. Consultation with a hematologist may be appropriate.
  • Individuals with AT deficiency may have heparin resistance, and, if they require anticoagulation, it may be necessary to use a nonheparin anticoagulant and/or to provide AT replacement along with appropriate doses of heparin. Refer to the UpToDate topic on AT deficiency for details.
  • Routine obstetrical care includes mechanical thromboprophylaxis and/or anticoagulation for women undergoing cesarean delivery. Routine postoperative care includes thromboprophylaxis in most cases; individuals with thrombophilia may require more aggressive prophylaxis than those without. Refer to UpToDate for indications for thrombophilia testing and other aspects of obstetric and postoperative management.
  • Routine perioperative care includes thromboprophylaxis based on the procedure-associated risk of VTE.
  • Atypical sites of VTE include mesenteric, portal, and cerebral veins.
VTE: venous thromboembolism; PE: pulmonary embolism; DVT: deep vein thrombosis; FVL: factor V Leiden mutation; PS: protein S; AT: antithrombin.
* Atypical sites of VTE include mesenteric, portal, and cerebral veins.
¶ All individuals with inherited thrombophilia should have education regarding signs and symptoms of VTE, attention to prophylaxis in high-risk situations (eg, certain surgeries, pregnancy, and postpartum for some women), risk assessment for first-degree relatives, and, for women, avoidance of estrogen-containing contraceptives.
Δ Routine care includes appropriate thromboprophylaxis for high-risk surgical procedures based on the procedure-associated risk of VTE.
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