Type 1 vWD diagnostic criteria
ISTH and HSC criteria for diagnosis of type 1 von Willebrand disease
|Category||ISTH criteria (2005) 1||HSC criteria (2000) 2|
|Type 1 vWD||All three of the following:||Both of the following:|
|Possible type 1 vWD||Laboratory tests compatible with type 1 vWD, and ONE of:||Any measurement of VWF:Ag or VWF:RCo below ABO-adjusted 95% lower conﬁdence limit|
Provisional criteria for the diagnosis of type 1 von Willebrand disease by a working party of the International Society on Thrombosis and Haemostasis (ISTH) were published in 2005,1 and criteria from the Hospital for Sick Children (HSC) in Toronto were published in 2000.2
The ISTH criteria are significantly more stringent than HSC criteria, although neither classify the majority of children presenting with bleeding symptoms and low vWF levels as having type 1 vWD.3 A separate category of ‘low von Willebrand factor’ has been suggested for those with reduced levels of vWF, but who do not meet laboratory criteria for definite or possible type 1 vWF.4
Alternative published criteria for the diagnosis of type 1 vWD include:
- 2008 National Heart, Lung, and Blood Institute (NHLBI) guidelines, which utilise a cut-off of vWF:Ag and vWF:RCO levels of < 30 IU/dL on at least two occasions.5
- 2004 UK Haemophilia Doctors Organisation (UKHDO) guidelines, which utilise more relaxed cut-off of vWF:Ag and vWF:RCO levels < 50 IU/dL on at least two occasions.6
- Sadler JE, Rodeghiero F; ISTH SSC Subcommittee on von Willebrand Factor. Provisional criteria for the diagnosis of VWD type 1. Journal of Thrombosis and Haemostasis 2005; 3: 775–7
- Dean JA, Blanchette VS, Carcao MD, Stain AM, Sparling CR, Siekmann J et al. von Willebrand disease in a pediatric-based population–comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor/collagen-binding assay. Thrombosis and Haemostasis 2000; 84: 401-9
- Hyatt SA, Wang W, Kerlin BA and O’Brien SH. Applying diagnostic criteria for type 1 von Willebrand disease to a pediatric population. Pediatric Blood and Cancer. 2009; 52: 102-7.
- Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. Journal of Thrombosis and Haemostasis 2006; 4: 2103–2114
- Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 14: 171-232.
- Laffan M, Brown SA, Collins PW, Cumming AM, Hill FG, Keeling D et al. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization. Haemophilia 2004; 10: 199-217.