How to Capture the Bleeding Phenotype in FXI-Deficient Patients.

Details

Serval ID
serval:BIB_865161825E59
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
How to Capture the Bleeding Phenotype in FXI-Deficient Patients.
Journal
Hamostaseologie
Author(s)
Bertaggia Calderara D., Zermatten M.G., Aliotta A., Alberio L.
ISSN
2567-5761 (Electronic)
ISSN-L
0720-9355
Publication state
Published
Issued date
11/2020
Peer-reviewed
Oui
Volume
40
Number
4
Pages
491-499
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Factor XI (FXI) is a serine protease involved in the propagation phase of coagulation and in providing clot stability. Several mutations in the F11 gene lead to FXI deficiency, a rare mild bleeding disorder. Current laboratory methods are unable to assess bleeding risk in FXI-deficient patients, because the degree of bleeding tendency does not correlate with plasma FXI activity as measured by routine coagulometric aPTT-based assays. Bleeding manifestations are highly variable among FXI-deficient patients and FXI replacement therapy can be associated with an increased thrombotic risk. A correct evaluation of the patient hemostatic potential is crucial to prevent under- or overtreatment. In recent years, different research groups have investigated the use of global coagulation assays as alternative for studying the role of FXI in hemostasis and identifying the clinical phenotype of FXI deficiency. This brief review article summarizes the main features of coagulation factor XI and its deficiency and resumes the principle axes of research and methods used to investigate FXI functions.
Pubmed
Web of science
Open Access
Yes
Create date
09/10/2020 13:42
Last modification date
23/11/2020 6:24
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