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 Printable version |
Factor XI deficiency |
Hématologie. Volume 16, Number 4, 284-92, juillet-août 2010, Revue
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 Résumé
Article gratuit
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Author(s) : Emmanuelle de Raucourt, Frédéric Bauduer, Brigitte Pan-Petesch, Jenny Goudemand |
Summary : FXI deficiency is a rare bleeding disorder which has a particularly high incidence among Ashkenazi Jews. FXI deficiency is a mild bleeding disorder associated with site injury bleeding typically involving tissue at high fibrinolytic activity. Replacement therapy either with fresh frozen plasma or a factor XI concentrate is the mainstay of therapy. The poor correlation between FXI levels and the bleeding phenotype, the variability of the bleeding diathesis even in the same patient, and the potential complications of the treatments have caused difficulty in formulating concrete treatment recommendations. This review gives an overview of the structure, and the role of FXI in the coagulation pathway, the pathophysiology, the genetic basis, the clinical manifestations and the management of FXI deficiency. |
Keywords : factor XI deficiency, factor XI, rare bleeding disorder |
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