Table 1

Phenotypic classification of VWD

VWD typePathophysiologic mechanism
Partial quantitative deficiency of VWF and/or FVIII 
2 Qualitative defects of VWF 
    A ↓ platelet-dependent VWF function, lack of HMWM 
    B ↑ platelet-dependent VWF function, lack of HMWM 
    M ↓ VWF function, normal multimers 
    N ↓ VWF binding to FVIII, normal multimers 
Severe/complete deficiency of VWF and moderately severe decrease of FVIII 
VWD typePathophysiologic mechanism
Partial quantitative deficiency of VWF and/or FVIII 
2 Qualitative defects of VWF 
    A ↓ platelet-dependent VWF function, lack of HMWM 
    B ↑ platelet-dependent VWF function, lack of HMWM 
    M ↓ VWF function, normal multimers 
    N ↓ VWF binding to FVIII, normal multimers 
Severe/complete deficiency of VWF and moderately severe decrease of FVIII 

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