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Semin Thromb Hemost 2021; 47(02): 201-216
DOI: 10.1055/s-0041-1723799
Review Article

Type 2B von Willebrand Disease in Pregnancy: A Systematic Literature Review

Mona M. Makhamreh
1   Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania
,
Melissa L. Russo
2   Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Warren Alpert Medical School of Brown University, Women and Infants Hospital of Rhode Island, Providence, Rhode Island
,
Taylor Karl
3   Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania
,
Natalie Delgado
3   Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania
,
Katherine Lackritz
4   Department of Obstetrics and Gynecology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania
,
Daniel W. Skupski
5   Departments of Obstetrics and Gynecology, Weill Cornell Medicine, New York, New York, and New York Presbyterian-Queens, Flushing, New York
,
Huda B. Al-Kouatly
1   Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania
› Author Affiliations Funding None.
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Abstract

Our objective was to review the maternal characteristics and obstetric complications in women with type 2B von Willebrand disease (VWD). A systematic literature search was conducted using PubMed, Scopus, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov. We included all publications that addressed type 2B VWD in pregnancy. Our primary and secondary outcomes were incidence of postpartum hemorrhage (PPH) and incidence of thrombocytopenia in pregnancy. Two reviewers independently identified eligible studies and abstracted data including maternal characteristics, hematologic characteristics, treatment, and delivery outcomes. Twenty studies met inclusion criteria. There were 27 women (32 pregnancies) with type 2B VWD. Primary PPH was reported in 9/20 women (45%) and secondary PPH was reported in 6/13 women (46%). Thrombocytopenia in pregnancy was present in 27/28 women (96%); 23/27 women (85%) had platelet count <100 × 109/L, mean 33.7 ± 22.7 × 109/L. Factor concentrate treatment was administered before delivery (n = 16) and postpartum (n = 18), some women received both. Seventeen deliveries required blood products postpartum with 13/17 (76%) platelet transfusions and 6/17 (35%) red blood cell transfusions. No maternal mortality was reported. Women with type 2B VWD have significant morbidity in pregnancy related to high incidence of severe thrombocytopenia and primary and secondary PPH.

Keywords

von Willebrand disease - type 2B - pregnancy - delivery - postpartum hemorrhage

Authors' Contributions

M.M.M. and T.K. contributed toward the acquisition of data. M.M.M., T.K., N.D., K.L., M.L.R., D.W.S, and H.B.A. did the analysis and interpretation of data, drafted the manuscript and finally approved the version to be published. They also agreed to be accountable for all aspects of the study.


Note

The work was presented at American College of Obstetricians and Gynecologists Annual Clinical and Scientific Meeting, poster presentation April 25th, 2020, Seattle, WA—presented virtually due to COVID-19.




Publication History

Article published online:
26 February 2021

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