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Factor V Leiden

Key sources
The following summarized guidelines for the evaluation and management of factor V Leiden are prepared by our editorial team based on guidelines from the Pathway (Pathway 2023), the American Society of Hematology (ASH 2023; 2018), the British Society for Haematology (BSH 2022), the European Society for Vascular Surgery (ESVS 2021; 2017), the American Association for the Study of Liver Diseases (AASLD 2021), the International Federation of Gynecology and Obstetrics (FIGO 2021), the American College of Gastroenterology (ACG 2020), the European Stroke Organisation (ESO 2017), the European Society for Trauma and Emergency Surgery (ESTES 2016), the Anticoagulation Forum (AF 2016), the European Association for the Study of the Liver (EASL 2016), the Society for Vascular Surgery (SVS 2014), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014), the Central European Vascular Forum (CEVF/IUA/IUP 2012), the British Committee for Standards In Haematology (BCSH 2012), the American College of Chest Physicians (ACCP 2012), and the American Heart Association (AHA/ASA 2011).
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Guidelines

1.Screening and diagnosis

Indications for screening, general principles, BSH: do not obtain routine thrombophilia testing in first-degree relatives of patients with a history of VTE.
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  • Indications for screening (minor provoking risk factors)

  • Indications for screening (before combined oral contraceptives)

  • Indications for screening (before hormonal therapy)

  • Indications for screening (pregnancy)

  • Indications for testing (first episode of thrombosis)

  • Indications for testing (DVT)

  • Indications for testing (cerebral vein thrombosis)

  • Indications for testing (mesenteric vein thrombosis)

  • Indications for testing (portal vein thrombosis)

  • Indications for testing (Budd-Chiari syndrome)

  • Indications for testing (superficial vein thrombosis)

  • Indications for testing (unusual site venous thrombosis)

  • Indications for testing (recurrent thrombosis)

  • Indications for testing (arterial thrombosis)

2.Medical management

Management of venous thromboembolism, general principles: manage VTE in FVL mutation carriers according to current published guidelines on VTE (including DVT, PE, cerebral venous thrombosis, as well as thrombosis in unusual sites).
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  • Management of VTE (DVT)

  • Management of VTE (cerebral vein thrombosis)

  • Management of VTE (mesenteric vein thrombosis)

  • Management of VTE (portal vein thrombosis)

3.Specific circumstances

Pregnant patients, screening, BSH: do not obtain screening for heritable thrombophilias in patients with pregnancy complications, such as recurrent miscarriage or adverse pregnancy outcomes.
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  • Pregnant patients (antepartum)

  • Pregnant patients (postpartum)