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Blood Rev. 2018 Jul;32(4):272-279. doi: 10.1016/j.blre.2018.01.002. Epub 2018 Apr 20.

Direct oral anticoagulant use in patients with thrombophilia, antiphospholipid syndrome or venous thrombosis of unusual sites: A narrative review.

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Service de Médecine Vasculaire et Thérapeutique, CHU de St-Etienne, Saint-Etienne, France; INSERM, UMR1059, Equipe Dysfonction Vasculaire et Hémostase, Université Jean-Monnet, F-42055 Saint-Etienne, France; INSERM, CIC-1408, CHU Saint-Etienne, F-42055 Saint-Etienne, France. Electronic address:
Internal Medicine and Vascular Medicine Department, Charles Nicolles Hospital, Rouen, France.
Neurology Department, University Hospital of Dijon Burgundy, France; Dijon Stroke Registry, EA4184, Medical School of Dijon, University of Burgundy, France.
Cardiology Department, Catholic Institute Hospitals Group of Lille, Medicine University, Catholic University of Lille, Lille, France.
Department of Cardiology, Hospital Henri Duffaut, Avignon, France.
Cardiology Office, F-67270 Hochfelden, France.
Rythmology Interventionnel Department, Henri Mondor Hospital, Creteil, France.
Department of Cardiology - Anticoagulation Clinic (CREATIF) - Inserm U942, Lariboisiere Hospital, AP-HP, Paris Diderot University, Sorbonne Paris Cité, Paris, France.
University of Occidentale Brittany, EA 3878 GETBO, Brest, France; Department of Internal Medicine and Pneumology, Hospital de la Cavale Blanche, Brest, France.


Direct oral anticoagulants (DOACs) are indicated in the treatment and prevention of venous thromboembolism (VTE). However, the use of DOACs in unusual VTE, including cerebral venous thrombosis (CVT) and splanchnic venous thrombosis (SVT), and in patients with biological thrombophilia including minor thrombophilia (Factor V Leiden and prothrombin G20210A), major innate thrombophilia (protein C and S deficiency, and antithrombin) and major acquired thrombophilia (antiphospholipid syndrome [APS]), remains controversial due to the paucity of available data. There are some reports of DOACs use in the initial treatment or long-term maintenance of patients with either CVT or SVT, but their efficacy remains unclear. The efficacy of DOACs may be suitable in patients with biological minor or major thrombophilia. The use of DOACs for the long-term maintenance of patients with APS is more contentious. Randomized clinical trials, which are currently underway, should offer definitive insight into the efficacy and safety profiles of DOACs in these patient populations.


Antiphospholipid syndrome; Cerebral venous thrombosis; Direct oral anticoagulants; Inherited thrombophilia; Splanchnic venous thrombosis

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