Semin Thromb Hemost 2016; 42(07): 732-740
DOI: 10.1055/s-0036-1587685
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Management of Factor XI Deficiency in Pregnancy

Joanna Davies
1   Department of Obstetrics and Gynaecology, The Royal Free Hospital, London, United Kingdom
,
Rezan Kadir
1   Department of Obstetrics and Gynaecology, The Royal Free Hospital, London, United Kingdom
› Author Affiliations
Further Information

Publication History

Publication Date:
03 October 2016 (online)

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.

 
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