Vol.:(0123456789) 1 3 Internal and Emergency Medicine https://doi.org/10.1007/s11739-019-02186-1 CE - RESEARCH LETTER TO THE EDITOR Direct oral anticoagulants in factor VII defciency patient Fulvio Pomero 1  · Laura Spadafora 2  · Salvatore D’Agnano 3  · Francesco Dentali 4  · Luigi Maria Fenoglio 5 Received: 27 June 2019 / Accepted: 26 August 2019 © Società Italiana di Medicina Interna (SIMI) 2019 Dear Sir, Factor VII (FVII) is a vitamin K-dependent coagulation factor synthesized in the liver which plays a major role in the coagulation extrinsic pathway which is initiated at tis- sue damage sites, following the formation of a complex between activated FVII (FVIIa) and tissue factor. Plasma levels range around 0.35–0.60 mg/L (for a normal coagulant activity comprised between 70 and 140%), which is 10 times less than other vitamin K-dependent factors. Its half-life is extremely short (4–6 h) [1]. Inherited FVII defciency, with an estimated prevalence of 1:300,000 population in Euro- pean countries, is the most common among the rare congeni- tal coagulation disorders, characterized by autosomal reces- sive inheritance. As with other forms of hemophilia, FVII defciency can also be caused by medications such as vita- min K antagonists (VKA), medical conditions or malabsorp- tion [2, 3]. Suspicion of FVII defciency arises in presence of a reduced FVII coagulant activity (FVII:C), a prolonged prothrombin time and an elevated international normalized ratio (INR) in the setting of normal liver function and a nor- mal activated partial thromboplastin time [1]. Clinical phe- notypes correlate poorly with FVII activity levels although hemorrhage rarely appears when FVII:C is above 30% and complete absence of FVII in plasma is usually incompat- ible with life and individuals die shortly after birth due to severe hemorrhage [4]. Nevertheless, venous and arterial thrombotic events have been reported in 3–4% of patients with FVII defciency. Thus, in some cases, “antithrombotic efect” of FVII defciency seems to be overwhelmed by the presence of thrombotic risk factors underlying the need of an antithrombotic prophylaxis even in these patients [5]. However, it is not well established how to manage the anti- coagulation therapy and the available evidences are based only on few case reports. Here, we describe a case of FVII defciency patient afected by atrial fbrillation (AF) treated with a direct oral anticoagulant (DOAC). In October 2017, an 89-year-old woman presented to the emergency depart- ment complaining of acute post-prandial pain in peri-umbil- ical region. On past medical history, she reported factor VII defciency, diagnosed at the age of 62 at the time visiting an anesthesiologist for a planned intervention of cholecystec- tomy. On this occasion, INR was 2 and the assays of VK- dependent factors showed a reduced FVII:C of 15%; at the time the patient was not taking any therapy. In 2011, she was hospitalized for acute heart failure caused by hypertension and AF: the physicians did not prescribe anticoagulants for hemorrhagic risk due to FVII defciency. In 2015, she was admitted to Neurology for a cerebellar ischemic stroke and was in treatment with clopidogrel at discharge. The patient had no other comorbidities including diabetes or dyslipi- demia; she regularly took furosemide, bisoprolol and clopi- dogrel. She weighed 58 kg, and was 161 cm high (body mass index 22.4). Upon physical examination, the patient was awake, alert and oriented to time, person, place and situation. Her blood pressure was 120/90 mmHg, heart rate of 90 beats/min with irregular rhythm. The abdomen was soft, slightly tender in the lower quadrants. Laboratory test showed increased white blood cells (12,170/uL), elevated INR (2.4), while C-reactive protein, amylase and troponin were within normal range. Creatinine was 0.89 mg/dl, ala- nine and aspartate transaminase were 27 IU/L and 33 IU/L, respectively. Fibrinogen was 2.4 g/L, D-dimer was 0.8 ug/ ml, aPTT was 30 s, and FVII:C was reduced to 10%. Her ECG showed atrial fbrillation. A contrast-enhanced CT scan was then performed, confrming the clinical suspicion of acute mesenteric ischemia: CT showed an occlusion of * Fulvio Pomero fulviopomero@yahoo.it 1 U.O. Medicina Interna, Ospedale S. Lazzaro, Via P. Belli 26, 12051 Alba, Italy 2 Emergency Department, S. Paolo Hospital, Savona, Italy 3 Specialty Training in Internal Medicine, University of Turin, Turin, Italy 4 Department of Clinical Medicine, University of Insubria, Varese, Italy 5 Department of Internal Medicine, ASO S. Croce E Carle, Cuneo, Italy