ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease.

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 Von Willebrand disease (VWD) is an inherited bleeding disorder. There are evidence based guidelines from various societies like American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF) and the World Federation of Hemophilia (WFH) for the diagnosis of VWD.

The Outcomes and Implementation Research unit at the University of Kansas Medical Centre (KUMC), supported the guideline-development process, including performing or updating systematic evidence reviews up to 8 January 2020. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE Evidence- to- Decision frameworks to assess evidence and make recommendations.

The panel agreed on 11 recommendations:

 


Figure 1 shows the diagnostic algorithm for VWD

Recommendation 1

For patients with a low probability of VWD (eg, seen in the primary care setting), the panel recommends using a validated BAT as an initial screening test to determine who needs specific blood testing over non-standardized clinical assessment.

Recommendation 2

For patients with an intermediate probability of VWD (eg,referred to a hematologist), the panel suggests against relying on a BAT to decide whether to order specific blood testing.

Recommendation 3

For patients with a high probability of VWD (eg, affected first degree relative), the panel recommends against relying on a BAT to decide whether to order specific blood testing.

Recommendation 4

The panel suggests newer assays that measure the platelet binding activity of VWF (eg, VWF:GPIbM,VWF:GPIbR) over the VWF:RCo assay (automated or nonautomated) for the diagnosis of VWD.

 Recommendation 5

The panel suggests reconsidering the diagnosis as opposed to removing the diagnosis for patients with previously confirmed type 1 VWD who now have VWF levels that have normalized with age.

 Recommendation 6

The panel recommends a VWF level of 0.30 IU/mL regardless of bleeding, and for patients with abnormal bleeding, a VWF level of 0.50 IU/mL to confirm the diagnosis of type 1 VWD.

Recommendation 7

The panel suggests against using the VWFpp/VWF:Ag (ratio of VWF propeptide to antigen) and rather using a desmopressin trial with 1- and 4-hour postinfusion blood work to confirm increased VWF clearance for patients with VWD suspected of type 1C should be used.


Fig 2  shows the algorithm for diagnosis for type 2b VWD 



Figure 3 shows the algorithm for diagnosis for type 2N VWD 

Recommendation 8

The panel suggests against a platelet-dependent VWF activity/VWF:Ag ratio cutoff of 0.5 and rather using a higher cutoff of 0.7 should be used to confirm type 2 VWD (2A,2B, or 2M) for patients with an abnormal initial VWD screen.

 Recommendation 9

The panel suggests either VWF multimer analysis or VWF:CB/VWF:Ag (ratio of VWF collagen binding to antigen) to diagnose type 2 VWD for patients suspected of type 2A, 2B, or 2M in need of additional testing.

Recommendation 10

The panel suggests targeted genetic testing over low-dose RIPA to diagnose type 2B VWD for patients suspected of type 2A or 2B in need of additional testing

Recommendation 11

The panel suggests using either VWF:FVIIIB or targeted genetic testing (when available) for patients with suspected type 2N VWD in need of additional testing.

How to use these guidelines

These guidelines are primarily intended to help clinicians make decisions about diagnostic and treatment alternatives. Other purposes are to inform policy, education, and advocacy, and to state future research needs. They may also be used by patients. These guidelines are not intended to serve or be construed as a standard of care.


References:

James et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von

Willebrand disease. Blood Advances. 2021:5(1).

                                                                                                          By Dr. Priyavadhana

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