VASCERN HHT Reference Centre and Genetic Department, Hospices Civils de Lyon, Lyon, France; Univ. Grenoble Alpes, Inserm, CEA, Laboratory Biology of Cancer and Infection, F-38000, Grenoble, France.
VASCERN HHT Reference Centre, Imperial College Healthcare National Health Service Trust, London, UK; National Heart and Lung Institute, Imperial College London, UK.
Eur J Med Genet. 2022 Oct;65(10):104575. doi: 10.1016/j.ejmg.2022.104575. Epub 2022 Aug 5.
Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular multisystemic disease that leads to epistaxis, anaemia due to blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver and brain. HHT prevalence is estimated at 1/6000, i.e. around 85,000 European citizens, and is served by the European Reference Network for Rare Multisystemic Vascular Diseases (VASCERN). HHT treatments depend on clinical manifestations, and span multiple different medical, surgical and interventional disciplines. Separate to local treatments in the nose, in severe settings, intravenous bevacizumab has been proposed as treatment option, and the purpose of the current article is to assess the use of intravenous bevacizumab in patients with HHT in 2022 according to available data.
遗传性出血性毛细血管扩张症（HHT）是一种罕见的血管多系统疾病，可导致鼻出血、失血引起的贫血以及肺部、肝脏和大脑等器官的动静脉畸形（AVMs）。HHT 的患病率估计为 1/6000，即约 85000 名欧洲公民，由欧洲罕见多系统血管疾病参考网络（VASCERN）提供服务。HHT 的治疗方法取决于临床表现，涵盖了多个不同的医学、外科和介入学科。除了在鼻子上进行局部治疗外，在严重情况下，还提出了静脉注射贝伐珠单抗作为治疗选择，本文的目的是根据现有数据评估 2022 年静脉注射贝伐珠单抗在 HHT 患者中的使用情况。
