Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 16, 2015; 3(4): 330-337
Published online Apr 16, 2015. doi: 10.12998/wjcc.v3.i4.330
Bleeding and clotting in hereditary hemorrhagic telangiectasia
Christopher Dittus, Michael Streiff, Jack Ansell
Christopher Dittus, Section of Hematology and Medical Oncology, Boston University Medical Center, Boston, MA 02118, United States
Michael Streiff, Department of Medicine, Johns Hopkins University, Baltimore, MD 21287, United States
Jack Ansell, Hofstra North Shore-LIJ School of Medicine, Hofstra University, Hempstead, NY 11549, United States
Author contributions: All authors contributed to this manuscript.
Conflict-of-interest: Dr. Dittus has no conflicts to report. Dr. Streiff has received research funding from Sanofi-Aventis, Bristol Myers Squibb and Portola, honoraria for CME lectures from Sanofi-Aventis and Ortho-McNeil, consulted for Sanofi-Aventis, Eisai, Daiichi-Sankyo, Boehringer-Ingelheim and Janssen HealthCare and has given expert witness testimony in various medical malpractice cases. Dr. Ansell serves as a consultant for Bristol Myers Squibb, Janssen, Boehringer Ingelheim, Pfizer, and Daiichi Sankyo Pharmaceuticals.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Christopher Dittus, DO, MPH, Section of Hematology and Medical Oncology, Boston University Medical Center, FGH Building, First Floor, 820 Harrison Avenue, Boston, MA 02118, United States.
Telephone: +1-917-7540887 Fax: +1-617-4141831
Received: October 23, 2014
Peer-review started: October 24, 2014
First decision: November 27, 2014
Revised: January 7, 2015
Accepted: January 18, 2015
Article in press: January 20, 2015
Published online: April 16, 2015

Hereditary hemorrhagic telangiectasia (HHT) is a relatively common inherited vascular disorder that was first described in 1864, and is notable for epistaxis, telangiectasia, and arterial venous malformations. While genetic tests are available, the diagnosis remains clinical, and is based on the Curacao criteria. Patients with HHT are at increased risk for both bleeding and clotting events. Because of these competing complications, hematologists are often faced with difficult clinical decisions. While the majority of management decisions revolve around bleeding complications, it is not infrequent for these patients to require anticoagulation for thrombosis. Any anticoagulation recommendations must take into account the bleeding risks associated with HHT. Recent reviews have found that HHT patients can be safely anticoagulated, with the most frequent complication being worsened epistaxis. Large clinical trials have shown that factor IIa and Xa inhibitors have less intracranial bleeding than warfarin, and basic coagulation research has provided a possible mechanism. This article describes the anticoagulation dilemma posed when a 62-year-old female patient with a history of bleeding events associated with HHT was diagnosed with a pulmonary embolism. The subsequent discussion focuses on the approach to anticoagulation in the HHT patient, and addresses the role of the new oral anticoagulants.

Keywords: Anticoagulation, Hereditary hemorrhagic telangiectasia, Hemorrhage, Thrombosis, Rivaroxaban, Apixaban, Dabigatran, Warfarin

Core tip: This article reviews an inherited disorder, hereditary hemorrhagic telangiectasia, in the context of a complicated clinical case. It highlights the problem of balancing the risks of bleeding and thrombosis, and raises the question of whether the new oral anticoagulants might provide safer therapy in such patients who need antithrombotic therapy.