Bibliographic Details
| Title: |
Natural history of patients with venous thromboembolism and hereditary hemorrhagic telangiectasia. Findings from the RIETE registry |
| Authors: |
Antoni Riera-Mestre, José María Mora-Luján, Javier Trujillo-Santos, Jorge Del Toro, José Antonio Nieto, José María Pedrajas, Raquel López-Reyes, Silvia Soler, Aitor Ballaz, Pau Cerdà, Manel Monreal, the RIETE Investigators |
| Source: |
Orphanet Journal of Rare Diseases, Vol 14, Iss 1, Pp 1-8 (2019) |
| Publisher Information: |
BMC, 2019. |
| Publication Year: |
2019 |
| Collection: |
LCC:Medicine |
| Subject Terms: |
Deep venous thrombosis, Hemorrhagic hereditary telangiectasia, Pulmonary embolism, Rare diseases, Venous thromboembolism, Medicine |
| More Details: |
Abstract Background Limited data exist about the clinical presentation, ideal therapy and outcomes of patients with hereditary hemorrhagic telangiectasia (HHT) who develop venous thromboembolism (VTE). Methods We 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). Results Of 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. Conclusions During anticoagulation for VTE in HHT patients, there were more bleeding events than VTE recurrences. Most bleeding episodes were non-major epistaxis. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
1750-1172 |
| Relation: |
http://link.springer.com/article/10.1186/s13023-019-1172-8; https://doaj.org/toc/1750-1172 |
| DOI: |
10.1186/s13023-019-1172-8 |
| Access URL: |
https://doaj.org/article/ccbe3e749c40412dbda72663415d0b61 |
| Accession Number: |
edsdoj.be3e749c40412dbda72663415d0b61 |
| Database: |
Directory of Open Access Journals |
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