Table 1.

Classification of Von Willebrand Disease

	Quantitative Deficiency of VWF
Type 1	Partial quantitative deficiency of VWF- autosomal dominant transmission (~60–70% of all cases)
Type 3	Virtually complete deficiency of VWF - autosomal recessive transmission (~ 1–2% of all cases)
	Qualitative Deficiency of VWF
Type 2	Qualitative deficiency of VWF (~ 25–30% of all cases)
Type 2A	Qualitative variants with decreased platelet-dependent function associated with the absence of high and intermediate-molecular–weight VWF multimers
Type 2B	Qualitative variants with increased affinity for platelet GpIb
Type 2M	Qualitative variants with decreased platelet-dependent function not caused by the absence of high-molecular–weight VWF multimers
Type 2N	Qualitative variants with markedly decreased affinity for FVIII

Note: Adapted with permission from Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006;4:2103–2114.15.