Direct Oral Anticoagulants in Patients with Thrombophilia: Challenges in Diagnostic Evaluation and Treatment

Direct oral anticoagulants (DOACs) or non-vitamin K oral anticoagulants (NOACs) are increasingly used in the prevention of recurrent venous thromboembolism (VTE), including that associated with thrombophilia. The efficacy of DOACs in thrombophilic patients, especially those with severe trombophilia or triple positive antiphospholipid syndrome (APS) with arterial thromboembolic events, remains controversial. Most case reports and case series indicate that DOACs are an attractive therapeutic option in the vast majority of these patients at high risk of recurrent VTE with more concerns raised in high-risk APS patients and these deficient in protein S (PS). Adherence to DOACs is of paramount importance in these patients. In this review we presented available data on the management of patients with thrombophilia using rivaroxaban, dabigatran or apixaban at standard doses. Moreover, we also demonstrated the overall effects of DOACs on coagulation tests, particularly those determined during thrombophilia screening such as lupus anticoagulant, antithrombin, protein C, PS, activated protein C ratio. Despite the paucity of data from randomized studies, the current evidence supports the use of DOACs in thrombophilia, especially those who prefer such treatment or have unstable anticoagulation with vitamin K antagonists or unacceptable adverse events while using these drugs.