Journal of Thrombosis and Thrombolysis ( IF 4 ) Pub Date : 2024-03-16 , DOI: 10.1007/s11239-024-02957-4 Omri Cohen , Gili Kenet , Sarina Levy-Mendelovich , Inna Tzoran , Benjamin Brenner , Cristina De Ancos , Patricia López-Miguel , José F. Varona , Judith Catella , Manuel Monreal , M. D. Adarraga , A. Alberich-Conesa , J. Aibar , A. Alda-Lozano , J. Alfonso , C. Amado , M. Angelina-García , J. I. Arcelus , A. Ballaz , R. Barba , C. Barbagelata , M. Barrón , B. Barrón-Andrés , F. Beddar-Chaib , A. Blanco-Molina , J. Criado , C. De Ancos , J. Del Toro , P. Demelo-Rodríguez , C. De Juana-Izquierdo , J. A. Díaz-Peromingo , A. Dubois-Silva , J. C. Escribano , C. Falgá , A. I. Farfán-Sedano , C. Fernández-Aracil , C. Fernández-Capitán , B. Fernández-Jiménez , J. L. Fernández-Reyes , M. A. Fidalgo , I. Francisco , C. Gabara , F. Galeano-Valle , F. García-Bragado , C. García-González , A. García-Ortega , O. Gavín-Sebastián , A. Gil-Díaz , A. M. Gómez , C. Gómez-Cuervo , E. Grau , L. Guirado , J. Gutiérrez , L. Hernández-Blasco , L. Jara-Palomares , M. J. Jaras , D. Jiménez , R. Jiménez , I. Jou , M. D. Joya , R. Lecumberri , J. M. León-Ramírez , J. L. Lobo , L. López-Jiménez , P. López-Miguel , J. J. López-Núñez , A. López-Ruiz , J. B. López-Sáez , A. Lorenzo , M. Lumbierres , O. Madridano , A. Maestre , P. J. Marchena , M. Marcos , M. Martín del Pozo , F. Martín-Martos , J. M. Maza , E. Mena , M. I. Mercado , J. Moises , M. Monreal , M. V. Morales , M. S. Navas , J. A. Nieto , M. J. Núñez-Fernández , M. Olid , L. Ordieres-Ortega , M. Ortiz , J. Osorio , S. Otálora , R. Otero , N. Pacheco-Gómez , J. Pagán , A. C. Palomeque , E. Paredes , P. Parra , J. M. Pedrajas , C. Pérez-Ductor , M. A. Pérez-Jacoiste , M. Pérez-Pinar , M. L. Peris , M. L. Pesce , J. A. Porras , R. Puchades , F. Rivera-Cívico , A. Rodríguez-Cobo , V. Rosa , M. Romero-Brugera , P. Ruiz-Artacho , N. Ruiz-Giménez , J. Ruiz-Ruiz , G. Salgueiro , T. Sancho , V. Sendín , P. Sigüenza , S. Soler , S. Suárez-Fernández , R. Tirado , C. Tolosa , A. Torrents-Vilar , M. I. Torres , J. Trujillo-Santos , E. Usandizaga , F. Uresandi , R. Valle , J. F. Varona , G. Vidal , A. Villalobos , P. Villares , C. Ay , S. Nopp , I. Pabinger , T. Vanassche , P. Verhamme , A. Verstraete , H. H. B. Yoo , A. C. Montenegro , S. N. Morales , J. Roa , J. Hirmerova , R. Malý , L. Bertoletti , A. Bura-Riviere , J. Catella , R. Chopard , F. Couturaud , O. Espitia , C. Grange , B. Leclercq , R. Le Mao , I. Mahé , F. Moustafa , L. Plaisance , G. Sarlon-Bartoli , P. Suchon , E. Versini , S. Schellong , B. Brenner , N. Dally , I. Tzoran , P. Sadeghipour , F. Rashidi , G. Barillari , M. Basaglia , F. Bilora , B. Brandolin , M. Ciammaichella , D. Colaizzo , F. Dentali , P. Di Micco , E. Grandone , E. Imbalzano , C. Marcon , R. Pesavento , A. Poz , P. Prandoni , C. Siniscalchi , B. Taflaj , A. Tufano , A. Visonà , B. Zalunardo , R. Paluga , A. Skride , D. Kigitovica , S. Fonseca , R. Marques , J. Meireles , S. Pinto , M. Bosevski , M. Trajkoca , M. Zdraveska , H. Bounameaux , L. Mazzolai , A. Aujayeb , J. A. Caprini , I. Weinberg , H. M. Bui , Manuel Monreal , Paolo Prandoni , Benjamin Brenner , Dominique Farge-Bancel , Raquel Barba , Pierpaolo Di Micco , Laurent Bertoletti , Sebastian Schellong , Inna Tzoran , Abilio Reis , Marijan Bosevski , Henri Bounameaux , Radovan Malý , Peter Verhamme , Joseph A. Caprini , Hanh My Bui ,
While direct oral anticoagulants (DOACs) are frequently used to treat venous thromboembolism (VTE), the outcomes of patients with inherited thrombophilia (IT) receiving DOACs for VTE remain understudied. We used data from the international RIETE registry to compare the rates of VTE recurrences, major bleeding, and mortality during anticoagulant treatment in VTE patients with and without IT, grouped by the use of DOACs or standard anticoagulant therapy. Among 103,818 enrolled patients, 21,089 (20.3%) were tested for IT, of whom 8422 (39.9%) tested positive: Protein C deficiency 294, Protein S deficiency 726, Antithrombin deficiency 240, Factor V Leiden 2248, Prothrombin gene mutation 1434, combined IT 3480. Overall, 14,189 RIETE patients (6.2% with IT) received DOACs, and 89,629 standard anticoagulation (8.4% with IT), mostly with heparins followed by vitamin K antagonists. Proportions of patients receiving DOACs did not differ between IT-positive and IT-negative patients. Rates of VTE recurrence on anticoagulant treatment were highest in patients with AT deficiency (P < 0.01). Rates of on-treatment major bleeding and all-cause mortality were lowest among patients with Factor V Leiden (FVL) or PT G20210A mutations, compared with patients who tested negative. Patients with IT who received DOACs had lower rates of major bleeding than those receiving standard anticoagulation. Excluding FVL and Protein S deficiency, patients with IT had lower rates of VTE recurrence with DOACs than with standard anticoagulation. DOACs are equally safe and effective in VTE patients with IT, with lower bleeding rates than those on standard anticoagulation.
中文翻译：
直接口服抗凝剂治疗的遗传性血栓形成倾向患者静脉血栓栓塞的结果：来自 RIETE 登记处的见解
虽然直接口服抗凝剂 (DOAC) 经常用于治疗静脉血栓栓塞 (VTE)，但接受 DOAC 治疗 VTE 的遗传性血栓形成倾向 (IT) 患者的结局仍有待研究。我们使用国际 RIETE 登记处的数据来比较有或没有 IT 的 VTE 患者在抗凝治疗期间的 VTE 复发率、大出血和死亡率，并按使用 DOAC 或标准抗凝治疗进行分组。在 103,818 名入组患者中，21,089 名（20.3%）接受了 IT 检测，其中 8422 名（39.9%）检测呈阳性：蛋白 C 缺乏 294、蛋白 S 缺乏 726、抗凝血酶缺乏 240、因子 V Leiden 2248、凝血酶原基因突变 1434，合计IT 3480。总体而言，14,189 名 RIETE 患者（6.2% 接受了 IT）接受了 DOAC 治疗，89,629 名患者接受了标准抗凝治疗（8.4% 接受了 IT），其中大部分使用肝素，其次是维生素 K 拮抗剂。IT 阳性和 IT 阴性患者之间接受 DOAC 的患者比例没有差异。AT 缺乏患者抗凝治疗后 VTE 复发率最高（P < 0.01）。与检测呈阴性的患者相比，具有 V 因子 Leiden (FVL) 或 PT G20210A 突变的患者的治疗中大出血率和全因死亡率最低。接受 DOAC 治疗的 IT 患者的大出血发生率低于接受标准抗凝治疗的患者。排除 FVL 和 Protein S 缺乏症，接受 DOAC 治疗的 IT 患者的 VTE 复发率低于接受标准抗凝治疗的患者。DOAC 对合并 IT 的 VTE 患者同样安全有效，与标准抗凝治疗相比，出血率更低。
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