The clinical management of factor XI deficiency in pregnant women

Expert Rev Hematol. 2020 Jul;13(7):719-729. doi: 10.1080/17474086.2020.1772745. Epub 2020 Jun 12.

Abstract

Introduction: Factor XI (FXI) deficiency is associated with highly variable bleeding, including excessive gynecologic and obstetrical bleeding. Since approximately 20% of FXI-deficient women will experience pregnancy-related bleeding, careful planning and knowledge of appropriate hemostatic management is pivotal for their care.

Areas covered: In this manuscript, authors present our current understanding of the role of FXI in hemostasis, the nature of the bleeding phenotype caused by its deficiency, and the impact of deficiency on obstetrical care. The authors searched PubMed with the terms, 'factor XI', 'factor XI deficiency', 'women', 'pregnancy', and 'obstetrics' to identify literature on these topics. Expectations of pregnancy-related complications in women with FXI deficiency, including antepartum, abortion-related, and postpartum bleeding, as well as bleeding associated with regional anesthesia are discussed. Recommendations for the care of these women are considered, including guidance for management of prophylactic care and acute bleeding.

Expert commentary: FXI deficiency results in a bleeding diathesis in some, but not all, patients, making treatment decisions and clinical management challenging. Currently available laboratory assays are not particularly useful for distinguishing patients with FXI deficiency who are prone to bleeding from those who are not. There is a need for alternative testing strategies to address this limitation.

Keywords: Factor XI; bleeding; factor XI deficiency; hemostasis; pregnancy.

Publication types

  • Meta-Analysis
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Anesthesia
  • Blood Coagulation
  • Blood Coagulation Tests
  • Clinical Decision-Making
  • Disease Management
  • Disease Susceptibility
  • Factor XI Deficiency / blood
  • Factor XI Deficiency / diagnosis*
  • Factor XI Deficiency / etiology
  • Factor XI Deficiency / therapy*
  • Female
  • Hemostasis
  • Humans
  • Postpartum Hemorrhage / etiology
  • Postpartum Hemorrhage / prevention & control
  • Pregnancy
  • Pregnancy Complications, Hematologic / blood
  • Pregnancy Complications, Hematologic / diagnosis*
  • Pregnancy Complications, Hematologic / etiology
  • Pregnancy Complications, Hematologic / therapy*
  • Risk Assessment
  • Risk Factors