Summaries of Must-Read Clinical Literature, Guidelines, and FDA Actions
Screening Tool for VWD Family Members Evaluated
Haemophilia; ePub 2017 Feb 8; Faiz, Kaveney, et al
A modified screening tool for family members of people with von Willebrand disease (VWD) can be used to group reproductive age women for hemostatic evaluation, according to a study involving 94 individuals.
Participants included previously untested family members of VWD patients (n=41), previously diagnosed VWD patients (n=26), and controls (n=27). Investigators administered the screening tool and drew blood for CBC, ferritin, and VWF testing. Patients completed a pictorial blood assessment chart (PBAC) with menses. Among the results:
- The tool was positive in 32% of family members, 77% of VWD patients, and 19% of controls.
- Combined with low ferritin, the tool was positive in two-thirds of family members, 9 in every 10 VWD patients, and 44% of controls.
- Low ferritin and the tool resulted in sensitivity of 86% and negative predictive value of 93%.
- In the control group, negative predictive value was between 92% and 95% for the tool; this was also the case when the tool was combined with low ferritin or a PBAC.
Faiz A, Kaveney A, Guo S, Murphy S, Philipp C. Screening of female family members of von Willebrand disease patients: Utility of a modified screening tool in a high-risk population. [Published online ahead of print February 8, 2017]. Haemophilia. doi:10.1111/hae.13188.