Abstract
Management of factor XI (FXI) deficiency in pregnancy is complicated by lack of correlation
between FXI level and bleeding risk. Clinicians should be vigilant about the potential
for prolonged or excessive bleeding following miscarriage or termination of pregnancy,
or postpartum hemorrhage (PPH). A multidisciplinary approach along with an individual
care plan is recommended to prevent bleeding complications. Assessment of bleeding
history, FXI level, and global tests of hemostasis can aid management decisions regarding
hemostatic prophylaxis. The risk of PPH can be minimized by obstetric measures to
avoid uterine atony and genital trauma, in addition to provision of appropriate hemostatic
prophylaxis for labor and delivery. Women with FXI deficiency can be given the option
of regional anesthesia, provided that prior consideration has been given to assessment
of potential bleeding risk and appropriate treatment strategies are implemented. Antifibrinolytic
agents are effective for the majority of women with FXI deficiency, but those with
severe deficiency/phenotype require FXI concentrate. Recombinant activated factor
VII (rFVIIa) has also been used successfully to prevent bleeding in FXI deficiency.
However, all treatments should be used with caution in pregnancy due to thrombogenic
potential. Neonatal bleeding complications are rare in FXI deficiency; however, hemostatic
assessment and cover are important for invasive procedures such as circumcision.
Keywords
factor XI deficiency - pregnancy - delivery
