Korte, W., Poon, M., Iorio, A. et al. (1 more author) (2017) Thrombosis in inherited fibrinogen disorders. Transfusion Medicine and Hemotherapy, 44 (2). pp. 70-76. ISSN 1660-3796
Abstract
Although inherited fibrinogen disorders (IFD) are primarily considered to be bleeding disorders, they are associated with a higher thrombotic complication risk than defects in other clotting factors. Managing IFD patients with thrombosis is challenging as anticoagulant treatment may exacerbate the underlying bleeding risk which can be life-threatening. Due to the low prevalence of IFD, there is little information on pathophysiology or optimal treatment of thrombosis in these patients. We searched the literature for cases of thrombosis among IFD patients and identified a total of 128 patient reports. In approximately half of the cases, thromboses were spontaneous, while in the others trauma, surgery, and parturition contributed to the risk. The true mechanism(s) of thrombosis in IFD patients remain to be elucidated. A variety of anticoagulant treatments have been used in the treatment or prevention of thrombosis, sometimes with concurrent fibrinogen replacement therapy. There is no definite evidence that fibrinogen supplementation increases the risk of thrombosis, and it may potentially be effective in the treatment and prevention of both thrombosis and hemorrhage in IFD patients.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2017 S. Karger GmbH, Freiburg. This is an author produced version of a paper subsequently published in Transfusion Medicine and Hemotherapy. Uploaded in accordance with the publisher's self-archiving policy. |
Keywords: | Blood coagulation disorders; Inherited; Congenital; Fibrinogen; Hemorrhage; Thrombosis |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > Department of Infection, Immunity and Cardiovascular Disease The University of Sheffield > Sheffield Teaching Hospitals |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 22 Feb 2017 11:41 |
Last Modified: | 14 Mar 2018 01:38 |
Published Version: | https://doi.org/10.1159/000452864 |
Status: | Published |
Publisher: | Karger Publishers |
Refereed: | Yes |
Identification Number: | 10.1159/000452864 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:112603 |