Factor XI Deficiency - 26/10/21
, Jean Marie Connors, MD b Résumé |
Factor XI (FXI) deficiency (hemophilia C or Rosenthal disease) was first described in the 1950s in a multigenerational family experiencing bleeding related to surgery and dental procedures. Managing patients with FXI deficiency presents several challenges, including a lack of correlation of bleeding symptoms with FXI activity levels, the large volume of fresh frozen plasma required to achieve hemostatic FXI levels, lack of availability of FXI concentrate in certain regions of the world, and the inherent thrombotic risk associated with replacement therapy. This article summarizes presentation, diagnosis, and management of patients with FXI deficiency in a variety of clinical settings.
Le texte complet de cet article est disponible en PDF.Keywords : Factor XI deficiency, Hemophilia C, Rosenthal syndrome, Plasma thromboplastin antecedent deficiency
Plan
Vol 35 - N° 6
P. 1157-1169 - décembre 2021 Retour au numéro
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