The Evolving Dilemma of Factor XI in Pregnancy: Suggestions for Management

Abstract

A case of a patient with severe factor XI (FXI) deficiency who presented for her seventh labor and delivery is presented. The nature of FXI deficiency, its prevalence, and issues related to genetic screening are discussed. Published literature on the topic is reviewed, including criteria that were developed to assess bleeding, laboratory tools used to estimate bleeding risk, and available treatments. Within the context of this challenging clinical dilemma, specific recommendations are provided for the antepartum, intrapartum, and postpartum stages of pregnancy. These include recommendations that take into account both FXI levels and history of any abnormal bleeding. While there are effective treatments available, it is important to consider that institutional multidisciplinary protocols are needed to manage this complex disorder. More work is needed to define the best management protocols.

Management of severe factor XI deficiency in pregnancy: A case report

“… 10 As FXI deficiency is often phenotypically silent, the presence of incidental findings is not uncommon. 11 …”

“…There is contradictory data in the literature concerning FXI levels during pregnancy, but several subsequent studies in FXI-deficient patients report no significant change over the course of pregnancy, in contrast to factor VIII and von Willebrand, which increase relatively during pregnancy. 4 , 11 However, measurement of FXI levels during the first and third trimesters remains essential for hematologists and anesthetists to decide the appropriate hemostatic prophylaxis during labor and delivery. 6 In the opinion of English authors, epidural anesthesia should be avoided in the presence of bleeding symptoms or severe deficiency but is not absolutely contraindicated.…”

Obstetric and perioperative management of patients with factor XI deficiency: a retrospective observational study

“…Some evidence points to plasma replacement therapy during labor not being mandatory even for women with severe FXI deficiency, as observed by Salomon et al 9 Furthermore, some anesthesiologists may be reluctant to administer neuraxial anesthesia to healthy women with FXI deficiency and no bleeding history despite growing evidence pointing toward its safety. 10 , 11 , 12 , 13 …”