Von Willebrand Disease and Other Hemostatic Abnormalities in Women with a History of Postpartum Haemorrhage

Abstract

Background: Postpartum haemorrhage (PPH), is the main cause of maternal morbidity and mortality globally but it is far more important in non-developed countries. PPH represents 25% of all maternal deaths worldwide. von Willebrand disease (VWD) is a disease caused by quantitative or qualitative deficiency of the von Willebrand factor (VWF) and women with VWD are at increased risk of PPH. Our aim was to establish a probable association of severe PPH in women with a history of haemostatic abnormalities. Methods: An observational, controlled study of adult women with a history of one or more episodes of life-threatening severe PPH requiring treatment in an intensive care unit or >10 units of blood products during the 24-hour period after diagnosis and their controls. The following tests were performed: blood cell count, blood group, renal, viral, liver function and haemostatic tests, fibrinogen, activity of the plasma factors and specific test to diagnose and classify VWD. Findings: We included 124 women with PPH and their controls. A total of 133 PPH events were recorded. The median age at the first event was 25.5 years old. Results were significantly different between the groups in terms of fibrinogen concentration, VWF:Ag, VWF:RCo, and FVIII. A specific diagnosis was established in 69 (55.6) and 4 (3.2%) patients in the PPH group and controls, respectively. Of 61 patients with VWD, 57 had type 1, two had type 2A, and another two had type 2B. Other non-significant haemostatic deficiencies were found between the groups. Interpretation: Our results show a relationship between PPH and inherited haemostatic disorders. VWD was the most frequent diagnosis but it was unknown at the time of delivery in all patients. Appropriate and opportune diagnosis before pregnancy of inherited haemostatic disorders, particularly VWD, may be important to effectively prevent and treat PPH. Funding: None. Declaration of Interest: Karim Majluf-Cruz: no conflict of interest to declare. Liliana Anguiano-Robledo: no conflict of interest to declare. Claudia C. Calzada-Mendoza: no conflict of interest to declare. Jesus Hernandez-Juarez: no conflict of interest to declare. Manuel Moreno-Hernandez: no conflict of interest to declare. Victor Manuel Dominguez-Reyes: no conflict of interest to declare. Anahi Guadalupe Figueroa-Torres: no conflict of interest to declare. Patricia Gomez-Rosas: no conflict of interest to declare. Rodrigo Arreola-Diaz: no conflict of interest to declare. Maria Teresa Garcia-Lee: no conflict of interest to declare. Maria Tania Ricardo-Moreno: no conflict of interest to declare. Rosa Elena Sosa-Camas: no conflict of interest to declare. Jaime Garcia-Chavez: no conflict of interest to declare. Irma Isordia-Salas: no conflict of interest to declare. Abraham Majluf-Cruz: no conflict of interest to declare. Ethical Approval: The study protocol was approved by the Human Ethics Committees and the Medical Research Councils of our institutions. Informed written consent was obtained from all patients before enrollment in the study. All women received complete information about the aim of the study and explained that their participation was voluntary. Data obtained was made anonymous immediately after blood drawing.