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Abstract(s)
A trombofilia é uma condição hereditária e/ou adquirida na qual uma anomalia da hemóstase aumenta o risco de trombose, levando à formação de trombos vasculares venosos e/ou arteriais. As trombofilias hereditárias comumente aceites são a variante do Fator V de Leiden, a variante do gene da protrombina (G20210A), deficiência de proteína C, deficiência de proteína S e deficiência de antitrombina. As trombofilias adquiridas mais relevantes incluem a síndrome antifosfolípidica, hemoglobinúria paroxística noturna, neoplasias mieloproliferativas e a presença de uma mutação JAK2 na ausência de um fenótipo de neoplasias mieloproliferativas. As manifestações clínicas compreendem o tromboembolismo venoso (trombose venosa profunda e embolia pulmonar), abortos recorrentes e complicações obstétricas, e tromboses menos comuns tais como trombose venosa cerebral, trombose venosa esplâncnica, e o acidente vascular cerebral e acidente isquémico transitório. Os eventos trombóticos são uma causa significativa de mortalidade e morbilidade no mundo ocidental.
Com uma multiplicidade de novas abordagens farmacológicas surgindo ao nível da terapêutica antitrombótica, os anticoagulantes orais diretos (dabigatrano, rivaroxabano, apixabano e edoxabano) podem oferecer mais vantagens relativamente aos antivitamínicos K (varfarina e acenocumarol) no tratamento e prevenção das doenças tromboembólicas, tendo resultados comparáveis ou melhores ao nível da eficácia e segurança. No entanto, a evidência é escassa no que diz respeito ao uso de anticoagulantes orais diretos em trombofilias hereditárias ou adquiridas. Assim, a varfarina, um fármaco com 70 anos de experiência e apesar das desvantagens ao nível da monitorização e segurança, permanece o gold standard da terapêutica anticoagulante oral na prevenção secundária de eventos tromboembólicos na trombofilia.
O farmacêutico desempenha um papel primordial para garantir a adesão à terapêutica e o uso racional da terapêutica anticoagulante oral.
Thrombophilia is a condition either acquired or inherited in which an anomaly in the hemostasis enhances the risk of thrombosis, leading to the formation of both vascular and arterial clots. Inherited thrombophilia most commonly accepted are the variant on the factor V of Leiden, variant on protrombin gene (G20210A), protein C deficiency, protein S deficiency and deficiency of antithrombin. Whereas the most relevant amongst the thrombophilias acquired include antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, myeloproliferative neoplasms, and the presence of a JAK2 mutation in the absence of a myeloproliferative neoplasms phenotype. Clinical manifestations include venous thromboembolism (deep vein thrombosis and pulmonary embolism), recurring miscarriage and obstetrics complications, and less common thrombosis such as cerebral venous thrombosis, splanchnic venous thrombosis, and stroke and transient ischemic accident. Thrombotic events are a significant cause of both mortality and morbidity throughout the western world. With an upcoming multitudinous of new pharmaceutical approaches in terms of the antithrombotic therapy, the direct oral anticoagulants (dabigatran, rivaroxaban, apixaban and edoxaban) can provide more advantages in comparison to vitamin K antagonists (warfarin and acenocoumarol) in both treatment and prevention of thromboembolic diseases, delivering similar or even heightened efficiency and security results. However, the evidence of direct oral anticoagulants on inherited and acquired thrombophilias is limited. Thus, warfarin, a drug known up for 70 years, and despite its disadvantages in terms of monitoring and safety, remains the gold standard of oral anticoagulant therapy in the secondary prevention of thromboembolic events in thrombophilia. Pharmacists undertake a key role in guaranteeing not only the adherence to the therapy but the rational use of the oral anticoagulant therapy as well.
Thrombophilia is a condition either acquired or inherited in which an anomaly in the hemostasis enhances the risk of thrombosis, leading to the formation of both vascular and arterial clots. Inherited thrombophilia most commonly accepted are the variant on the factor V of Leiden, variant on protrombin gene (G20210A), protein C deficiency, protein S deficiency and deficiency of antithrombin. Whereas the most relevant amongst the thrombophilias acquired include antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, myeloproliferative neoplasms, and the presence of a JAK2 mutation in the absence of a myeloproliferative neoplasms phenotype. Clinical manifestations include venous thromboembolism (deep vein thrombosis and pulmonary embolism), recurring miscarriage and obstetrics complications, and less common thrombosis such as cerebral venous thrombosis, splanchnic venous thrombosis, and stroke and transient ischemic accident. Thrombotic events are a significant cause of both mortality and morbidity throughout the western world. With an upcoming multitudinous of new pharmaceutical approaches in terms of the antithrombotic therapy, the direct oral anticoagulants (dabigatran, rivaroxaban, apixaban and edoxaban) can provide more advantages in comparison to vitamin K antagonists (warfarin and acenocoumarol) in both treatment and prevention of thromboembolic diseases, delivering similar or even heightened efficiency and security results. However, the evidence of direct oral anticoagulants on inherited and acquired thrombophilias is limited. Thus, warfarin, a drug known up for 70 years, and despite its disadvantages in terms of monitoring and safety, remains the gold standard of oral anticoagulant therapy in the secondary prevention of thromboembolic events in thrombophilia. Pharmacists undertake a key role in guaranteeing not only the adherence to the therapy but the rational use of the oral anticoagulant therapy as well.
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Keywords
Anticoagulação oral Anticoagulantes orais diretos Antagonistas da vitamina k Trombofilia