Present day management of inherited bleeding disorders in pregnancy

Expert Rev Hematol. 2016 Oct;9(10):987-95. doi: 10.1080/17474086.2016.1216312. Epub 2016 Aug 2.


Introduction: How specifically to prevent post-partum haemorrhage (PPH) remains a challenge in pregnant women with an inherited bleeding disorder. There exists a morbidity of 5-10% of patients as well as increased mortality.

Areas covered: This review will survey the literature based on Medline review and various society monographs. Numerous societies have developed guidelines in hopes of reducing the risk of PPH. The guidelines are congruent in stating that the von Willebrand factor (VWF) /Factor VIII (FVIII) level must be > 50% to both permit safely epidural analgesia but also to prevent PPH. However, specific guidance is lacking in terms of how high and long a level should be achieved. Recent studies report a high rate of PPH in these treated patients despite aiming for levels > 50-100% suggesting that a postpartum post-replacement VWF/FVIII level of 50-100% is inadequate and follow up dosing should maintain higher levels then typically achieved per 'guidelines'. Expert commentary: Future studies to reduce PPH in women in the third trimester with levels < 50% should incorporate concurrent post-partum antifibrinolytic agent therapy and/or double utertonics and/or aiming for a higher trough factor level closer to 200% than 100% and maintaining such a level for several days postpartum.

Keywords: Post-partum hemorrhage; VWF-containing plasma-derived Factor VIII concentrate; hemophilia carrier; obstetrical hemorrhage; rare bleeding disorder; recombinant factor VIII; tranexamic acid; von Willebrand disease.

Publication types

  • Meta-Analysis
  • Review

MeSH terms

  • Adult
  • Blood Coagulation
  • Blood Coagulation Disorders, Inherited / diagnosis*
  • Blood Coagulation Disorders, Inherited / epidemiology
  • Blood Coagulation Disorders, Inherited / etiology
  • Blood Coagulation Disorders, Inherited / therapy*
  • Disease Management
  • Female
  • Hemophilia A / diagnosis
  • Hemophilia A / genetics
  • Hemophilia A / therapy
  • Heterozygote
  • Humans
  • Practice Guidelines as Topic
  • Pregnancy
  • Pregnancy Complications, Hematologic*
  • Risk Factors
  • von Willebrand Diseases / diagnosis
  • von Willebrand Diseases / genetics
  • von Willebrand Diseases / therapy