Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry
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静脉血栓栓塞和遗传性出血性毛细血管扩张症患者的自然史。
DOI：
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发表时间：
2019
影响因子：
3.7
通讯作者：
M. Monreal
中科院分区：
文献类型：
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作者：
A. Riera;J. M. Mora;J. Trujillo;J. del Toro;J. Nieto;J. Pedrajas;R. Lopez;S. Soler;A. Ballaz;P. Cerdà;M. Monreal
关于遗传性出血性毛细血管扩张症(HHT)患者发生静脉血栓栓塞症(VTE)的临床表现、理想治疗和预后的数据有限。方法利用RIETE注册表中的数据，根据最初表现为肺栓塞(PE)或深静脉血栓形成(DVT)，评估HHT患者的临床特征、治疗方法和临床结果。结果2009年2月至2019年1月在RIETE登记的51,375例急性VTE患者中，23例(0.04%)HHT14例(61%)最初表现为PE，9例(39%)仅表现为DVT。近一半(47.8%)的VTE患者存在VTE的危险因素。大多数PE和DVT患者接受低分子肝素治疗初期(分别为71%和100%)和长期治疗(分别为54%和67%)。在静脉血栓栓塞术中，出血事件的发生率(主要的2例，非主要的6例)远远超过静脉血栓栓塞术的复发率(复发的深静脉血栓栓塞术1)：每百人年出血量50.1次(95%可信区间：2 1.6-98.7)对6.26次复发(95%可信区间：0.31~30.9；p = 0.020)。1例大出血和3例非大出血为鼻出血。无一例患者死于出血。1例患者在诊断为急性PE后不久死亡。结论HHT患者在VTE抗凝治疗过程中，出血事件多于VTE复发。出血多为非重度鼻出血。
BackgroundLimited data exist about the clinical presentation, ideal therapy and outcomes of patients with hereditary hemorrhagic telangiectasia (HHT) who develop venous thromboembolism (VTE).MethodsWe used the data in the RIETE Registry to assess the clinical characteristics, therapeutic approaches and clinical outcomes during the course of anticoagulant therapy in patients with HHT according to initial presentation as pulmonary embolism (PE) or deep venous thrombosis (DVT).ResultsOf 51,375 patients with acute VTE enrolled in RIETE from February 2009 to January 2019, 23 (0.04%) had HHT: 14 (61%) initially presented with PE and 9 (39%) with DVT alone. Almost half (47.8%) of the patients with VTE had a risk factor for VTE. Most PE and DVT patients received low-molecular-weight heparin for initial (71 and 100%, respectively) and long-term therapy (54 and 67%, respectively). During anticoagulation for VTE, the rate of bleeding events (major 2, non-major 6) far outweighed the rate of VTE recurrences (recurrent DVT 1): 50.1 bleeds per 100 patient-years (95%CI: 21.6-98.7) vs. 6.26 recurrences (95%CI: 0.31–30.9; p = 0.020). One major and three non-major bleeding were epistaxis. No patient died of bleeding. One patient died shortly after being diagnosed with acute PE.ConclusionsDuring anticoagulation for VTE in HHT patients, there were more bleeding events than VTE recurrences. Most bleeding episodes were non-major epistaxis.