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Background: Hereditary and acquired thrombophilia are risk factors for venous thromboembolism (VTE). Whether testing helps in guiding management decisions is controversial. Objective: These evidence-based guidelines from the American Society of Hematology (ASH) intend to support decision-making about thrombophilia testing. Methods: ASH formed a multidisciplinary guideline panel covering clinical and methodological expertise and minimizing bias from conflicts of interest. The McMaster University GRADE Centre provided logistical support, performed systematic reviews, and created evidence profiles and evidence-to-decision tables. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used. Recommendations were subject to public comment. Results: The panel agreed on 23 recommendations regarding thrombophilia testing and associated management. Nearly all recommendations are based on very low certainty in the evidence due to modeling assumptions. Conclusions: The panel issued a strong recommendation against testing the general population before starting combined oral contraceptives (COC), and conditional recommendations for thrombophilia testing in the following scenarios: a) patients with VTE associated with non-surgical major transient or hormonal risk factors; b) patients with cerebral or splanchnic venous thrombosis, in settings where anticoagulation would otherwise be discontinued; c) individuals with a family history of antithrombin, protein C or protein S deficiency when considering thromboprophylaxis for minor provoking risk factors, and for guidance to avoid COC/HRT; d) pregnant women with a family history of high-risk thrombophilia types; e) patients with cancer at low or intermediate risk of thrombosis and with a family history of VTE. For all other questions, the panel provided conditional recommendations against testing for thrombophilia.

Background:Clinicians confront numerous practical issues in optimizing the use of anticoagulants to treat venous thromboembolism (VTE).Objective:These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians and other health care professionals in their decisions about the use of anticoagulants in the management of VTE. These guidelines assume the choice of anticoagulant has already been made.Methods:ASH formed a multidisciplinary guideline panel balanced to minimize potential bias from conflicts of interest. The McMaster University GRADE Centre supported the guideline development process, including updating or performing systematic evidence reviews. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess evidence and make recommendations, which were subject to public comment.Results:The panel agreed on 25 recommendations and 2 good practice statements to optimize management of patients receiving anticoagulants.Conclusions:Strong recommendations included using patient self-management of international normalized ratio (INR) with home point-of-care INR monitoring for vitamin K antagonist therapy and against using periprocedural low-molecular-weight heparin (LMWH) bridging therapy. Conditional recommendations included basing treatment dosing of LMWH on actual body weight, not using anti–factor Xa monitoring to guide LMWH dosing, using specialized anticoagulation management services, and resuming anticoagulation after episodes of life-threatening bleeding.