Table 1.

Summary of prioritized questions

	Population	Intervention	Comparator	Outcome
1	Patients suspected of VWD	Using a BAT to screen for VWD	Nonstandardized clinical assessment (not using a BAT)	Accurate VWD diagnosis
2	Patients suspected of VWD with negative/normal BAT (eg, men and children)	Blood testing	No blood testing	Accurate VWD diagnosis
3	Patients suspected of VWD	VWF:RCo (automated and nonautomated assays)	Newer assays that reflect the platelet-binding activity of VWF (eg, VWF:GPIbM, VWF:GPIbR).	Accurate VWD diagnosis/classification
4	Patients with a historic diagnosis of type 1 VWD but who now have normal VWF levels	Reconsider the diagnosis	Remove the diagnosis	Accurate VWD diagnosis
5	Patients with abnormal initial VWD screen (low VWF:Ag and/or platelet-dependent VWF activity) and suspected type 1 VWD	Diagnosis of type 1 VWD at VWF:Ag and/or platelet-dependent VWF activity <0.30 IU/mL	Diagnosis of type 1 VWD at VWF:Ag and/or platelet-dependent VWF activity <0.50 IU/mL	Accurate VWD diagnosis (to identify type 1 VWD)
6	Patients suspected of type 1C VWD	VWFpp/VWF:Ag	Desmopressin trial with 1- and 4-h blood work	Accurate VWD classification (to identify type 1C)
7	Patients with abnormal initial screen (low VWF:Ag and/or platelet-dependent VWF activity) and suspected of type 2 VWD	Platelet-dependent VWF activity/VWF:Ag ratio <0.5	Higher cutoff <0.7	Accurate VWD classification (to identify those suspected of having type 2A, 2B, or 2M in need of additional testing)
8	Patients suspected of type 2A, 2B, or 2M in need of additional testing	VWF multimer analysis	VWF:CB/VWF:Ag	Accurate VWD classification (to identify type 2A, 2B, or 2M)
9	Patients suspected of type 2A or 2B VWD	Low-dose RIPA	Targeted genetic testing	Accurate VWD classification (to identify type 2B)
10	Patients suspected of type 2N in need of additional testing	VWF:FVIII binding	Targeted genetic testing	Accurate VWD classification (to identify type 2N)