CASI CLINICI – Case Reports
Volume:
Biochimica Clinica, 2024; 48(2) e13-e17
Pubblicato on-line:
March 1, 2024
DOI:
10.19186/BC_2024.014
Difetti emostatici in un paziente in trattamento con anticoagulanti orali diretti
AUTORI
1Azienda Ospedaliero Universitaria Pisana- UOC Laboratorio Analisi Chimico Cliniche, Pisa
2Azienda Ospedaliero Universitaria Pisana - Centro Antitrombosi FCSA (Federazione Centri per la diagnosi e la Sorveglianza delle terapie Antitrombotiche) n.281, Pisa
3Azienda Ospedaliero Universitaria Pisana - SOD Patologia Clinica, Pisa
4Presidio Ospedaliero Felice Lotti - UOC Medicina Generale, Pontedera (PI)
5Presidio Ospedaliero Felice Lotti - UOC Laboratorio Analisi Chimico Cliniche VDE (Valdera) e AVC (Alta Val di Cecina), Pontedera (PI)
6Università di Firenze, Dipartimento di Medicina Sperimentale e Clinica, Firenze
7AOU Careggi - SOD Malattie Emorragiche, Firenze
ABSTRACT
Emostatic abnormalities in a patient treated with direct oral anticoagulants
This case report concerns a 68-year-old man with atrial fibrillation who was being treated with edoxaban, a direct oral anticoagulant, to prevent stroke. In the patient’s medical history, two episodes of post-surgical hemorrhagic diathesis were not investigated. He sought First Aid for intense asthenia and dark stool discharge. He presented with severe anemia (hemoglobin 40 g/L) and extended coagulation tests (prothrombin ratio 8.56; activated partial thromboplastin ratio 3.83), antral gastritis, and a gastric ulcer. The prolongation of both coagulation tests suggested a decrease in the activity of one of the common pathway factors.
The subsequent performance of a mixing test, which helps to distinguish clotting time prolongation due to a coagulation factor deficiency rather than to an inhibitor, allowed us to understand that the patient might be suffering from a factor congenital deficiency. Measurements of coagulation factors showed a severe reduction in factor V activity (1.1%). The search for specific inhibitors directed against factor V was negative, confirming the suspicion that it was a congenital deficiency of factor V, a rare autosomal recessive disorder.
Anticoagulant therapy and gastric ulcer made it possible to diagnose a rare congenital disease in an elderly man. The anticoagulant therapy was interrupted and a surgical closure of the left atrial appendage was established.
Before starting anticoagulant therapy, according to consolidated guidelines, a blood count, full coagulation panel, renal, and liver function must be evaluated. Very likely, the patient, affected by congenital factor V deficiency, started the anticoagulant therapy without an adequate evaluation of the hemostasis status.
BIBLIOGRAFIA
1. Asselta R, Peyvandi F. Factor V Deficiency. Semin Thromb Hemost 2009;35:382-9.
2. Mann KG, Kalafatis M. Factor V: a combination of Dr Jekyll and Mr Hyde. Blood 2003;101:20-30.
3. Owren PA. Parahaemophilia; Haemorragic diathesis due to absence of a previously unknown clotting factor. Lancet 1947;1:446-8.
4. Mannucci PM. Rare Inherited coagulation disorders In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.
5. Girolami A, Ruzzon E, Tezza F, Scandellari R, Vettore S, Girolami B. Arterial and venous thrombosis in rare congenital bleeding disorders: a critical review. Haemophilia 2006;12:345.
6. Levy Mendelovich S, Barg AA, Rosenberg N, Avishai E, Luboshitz J, Misgav M et al. Treatment tailoring for factor V deficient patients and perioperative management using global hemostatic coagulation assay. Blood Cells Mol Dis 2018;71:5-10.
7. Guida alla terapia antitrombotica – Raccomandazioni – FCSA Federazione Centri per diagnosi della trombosi e la Sorveglianza delle terapie Antitrombotiche, XXI Edizione 2023.
8. Steffel J, Collins R, Antz M, Cornu P, Desteghe L, Haeusler KG et al. 2021 European Heart Rhythm Association Practical Guide of the use of non-vitamin K antagonist oral anticoagulants in patients with atrial fibrillation. Eur Heart J 2021;23:1612-76.
9. Malcangi G, Coppola A, Marino R, Molinari AC, Santoro RC, Tagliaferri A. Gestione delle comorbilità nel paziente con emofilia. https://books.seedstm.com/index.php/seed/catalog/download/Gestione_comorbilita_emofilia/22/162-1?inline=1 (ultimo accesso: gennaio 2024)
10. Badescu MC, Badulescu OV, Butnariu LI et al. Current therapeutic approach to atrial fibrillation in patients with congenital hemophilia. J Pers Med 2022;12:519.
