Newly published results from a literature review-based study suggest that primary postpartum hemorrhage (PPH) is a persistent clinical challenge in patients with type 3 von Willebrand disease (VWD), even in those who received their diagnosis before pregnancy. The review was conducted by a team of researchers led by Mona Makhamreh, MD, from the Department of Obstetrics and Gynecology, at Thomas Jefferson University in Philadelphia, PA.

Type 3 VWD is the rarest form of the disease with affected individuals manifesting the most severe bleeding symptoms, when compared to other types of VWD. PPH is defined as blood loss of more than 500 milliliters (mL) following vaginal delivery or more than 1000 mL following cesarean delivery. When this amount of blood loss occurs within 24 hours of delivery it is known as primary PPH and when it occurs between 24 hours and 12 weeks after delivery it is defined as secondary PPH.

The broad systematic literature review performed by Makhamreh and her colleagues utilized PubMed, Scopus, and Cochrane Central Register of Controlled Trials to search for articles relevant to VWD and pregnancy and published between January 1982 and May 2019. They identified a total of 13 studies representing 28 pregnancies in 17 patients with type 3 VWD, all of which were diagnosed before pregnancy.

Factor replacement therapy was administered prior to delivery in 19 of the pregnancies and post-delivery in 26 of the pregnancies. PPH was reported in 48% (10/21) and secondary PPH was reported in 56% (5/9). Secondary PPH occurred between 7 and 22 days. In addition, none of the studies reported hysterectomies, intensive care unit admissions, or maternal mortality. The authors also reported that all 28 pregnancies resulted in live births at term.

“Our review highlights the maternal outcomes in patients with Type 3 VWD and the different approaches in management during pregnancy and delivery. Despite prior knowledge of this bleeding disorder, PPH was still a significant complication,” concluded the authors.

“Type 3 von Willebrand Disease in Pregnancy: A Systematic Literature Review,” was published online in the American Journal of Perinatology.

Source: Hematology Advisor, January 14, 2020