Type 1 vWD diagnostic criteria

ISTH and HSC criteria for diagnosis of type 1 von Willebrand disease

CategoryISTH criteria (2005) 1HSC criteria (2000) 2
Type 1 vWDAll three of the following:
Both of the following:
Possible type 1 vWDLaboratory tests compatible with type 1 vWD, and ONE of:
  • Significant muco­cutaneous bleeding symptoms, OR
  • Positive family history, or an appropriate vWF mutation
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