Annales de Biologie Clinique


Factor XI deficiency: actuality and review of the literature Volume 82, issue 2, March-April 2024

1 Laboratoire d’Hématologie, Centre Hospitalier de Niort , 40 avenue Charles de Gaulle, 79000 Niort
2 Laboratoire, Groupement de coopération sanitaire de Saintonge, Laboratoire inter-hospitalier de biologie médicale, Centres hospitaliers de Saint-Jean-d’Angély, Saintes, Royan et Jonzac, Saint-Jean-d’Angély, 18 avenue du port, 17400 St Jean d’Angély
3 Laboratoire d’Hématologie, CHU d’Angers, 4 rue Larrey, 49100 Angers
4 Laboratoire d’hématologie, Centre Hospitalier Universitaire d’Angers
5 Service d’Anesthésie, Clinique Pasteur, 222 Av. de Rochefort, 17200 Royan
* Correspondance M. Demoy

Although two clusters have been identified in France, constitutional factor XI deficiency is a rare disorder. Acquired factor XI deficiency is extremely rare. The management of factor XI deficiency is not staightforward because of the unpredictable bleeding tendency that does not clearly relate to the factor XI level. Other haemostastis parameters have to be taken into account to evaluate the bleeding tendency. We report the cases of a congenital factor XI deficiency, an acquired factor XI deficiency and a von Willebrand disease associated to a factor XI deficiency. On the other hand, some interferences can lead to underestimation of factor XI and we report the case of an interference by lupus anticoagulant. The objective of this review is to better understand how to manage a reduced factor XI level.