Significant gynecological bleeding in women with low von Willebrand factor levels

Abstract

Gynecological bleeding is frequently reported in women with von Willebrand disease (VWD). Low von Willebrand factor (VWF) may be associated with significant bleeding phenotype despite only mild plasma VWF reductions. The contribution of gynecological bleeding to this phenotype has yet to be described. The optimal clinical bleeding assessment tool (BAT) to evaluate bleeding remains unclear. Using a standardized approach to phenotypic assessment, we evaluated gynecological bleeding and directly compared the Condensed Molecular and Clinical Markers for the Diagnosis and Management of type 1 VWD (Condensed MCMDM-1 VWD) and International Society on Thrombosis and Haemostasis (ISTH) BAT scores in 120 women enrolled in the Low von Willebrand in Ireland Cohort study. Heavy menstrual bleeding (HMB) was reported in 89% of female participants; 45.8% developed iron deficiency. Using identical data, Condensed MCMDM-1 VWD menorrhagia domain scores were significantly lower than ISTH BAT scores (2 vs 3; < .0001), the discrepant results related to 40% of women not seeking medical consultation for HMB, reducing the sensitivity of the Condensed score. For those who reported HMB to physicians, the low VWF diagnosis was not expedited (age at diagnosis 34.2 vs 33.4 years in women failing to present; = .7). Postpartum hemorrhage (PPH) was self-reported in 63.5% of parous women (n = 74); 21.6% required transfusion, critical care, radiological, or surgical intervention. Our data demonstrate that gynecological bleeding is frequently reported in women with low VWF; despite pregnancy-related increases in plasma VWF levels, these women may experience PPH. Defining the optimal management approach for these patients requires further research. This trial was registered at www.clinicaltrials.gov as #NCT03167320.

Severe postpartum haemorrhage as first presenting symptom of an inherited bleeding disorder

“…In line with our results, one study also mentioned the value of a Bleeding Assessment Tool, as the only woman diagnosed with a bleeding disorder was also the only patient who reported more than two additional bleeding symptoms. 23 Based on these and our data, maintaining higher plasma VWF levels could be considered during the peripartum period, in addition to early administration of TXA. This might indicate that, although there is a physiological increase in plasma VWF levels during pregnancy, 9,22 some women with low VWF apparently still are at risk for PPH.…”

Laboratory monitoring during pregnancy and post‐partum hemorrhage in women with von Willebrand disease

“…For example, in a recent single‐center retrospective study of 86 pregnancies in 55 women with VWD, all patients underwent third trimester monitoring, yet the incidence of primary PPH was 6.9%, similar to our findings in a population with much lower rates of screening . One cause of high rates of PPH despite third trimester monitoring may be current guidelines that recommend VWF replacement to > 50%, although recent studies suggest we are undertreating women with VWD with this approach and should rather be aiming closer to the levels of 150% to 200% that occur postpartum in the general population …”

“…16 One cause of high rates of PPH despite third trimester monitoring may be current guidelines that recommend VWF replacement to > 50%, 5,18 although recent studies suggest we are undertreating women with VWD with this approach and should rather be aiming closer to the levels of 150% to 200% that occur postpartum in the general population. 11,[18][19][20] In conclusion, we found that third-trimester VWF monitoring was associated with a lower risk of PPH, but that testing was performed in only one-third of insured pregnant women with VWD in the United States despite expert recommendations. We also identified PPH events reported late (>2 weeks) in the postpartum period, and identified women presenting with HMB in the first 12 weeks after delivery.…”

A single‐centre study of management of pregnant women with von Willebrand disease

“…1 Women with VWD are at risk of a variety of bleeding complications during pregnancy owing to invasive prenatal procedures and the haemostatic challenge of delivery. 2 Retrospective studies found that women with VWD have also a higher rate of postpartum haemorrhage (PPH) and of transfusion at the time of delivery. 2,3 In the last decade, there has been increased research in women with inherited bleeding disorders including VWD.…”