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PVT


Perth Haematology :: Dr Steven Ward

von Willebrand's Disease

 

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nAutosomal dominant defect of vWF
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nBleeding usually mucosal, surgical
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nEpistaxis 60%; bruising 40%; menorrhagia 35%; gum bleeds 35%; GI bleed 10%
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nBleeding is variable in individual and within family
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nCommon - ? 1% of population
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nCan be hard to diagnose - vWF Ag
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nLevels fluctuate; vWF is an acute phase protein; levels vary with blood groups
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nAPTT and routine coag profile can be normal

vWD pdf File

 

DDAVP Therapy

Factor VIII Therapy


Classification of vWD

vWD:

Type 1

Type 2A

Type 2B

Type 2M

Type 2N

Type 3

 

Mild

Qualitative defect in vWF

Severe


Quantitative loss of VWF
70% of vWD cases
Reduced binding to platelet GP1. Reduced mutimers.
Enhanced binding to platelet GP1 - clear plats and multimers - low platelets
Reduecd GP1 binding and normal multimers
Reduced binding vWF to FVIII.
Homozygous loss of vWF. Rare.
Inheritance AD AD AD AD AD AR

FVIII

vWF Ag

vWF RiCoF

VWF CBA

N or
N or
↓↓
N or
N or
↓↓
N or
N or


N
N
N
↓↓
↓↓↓
↓↓↓
↓↓↓

vWF Multimers

N

ABN - loss of HMW

ABN - loss of HMW

N

N

NIL

RIPA

 

 

 

 

 

vWF:FVIII binding activity

 

 

 

 

 


Platelet-type vWD (pseudo-vWD)
AD inherited defect of plaelets. vWF is normal. Altered platelet GP1 with increased affinity for vWF - removes platelet aggregates and HMW multimers from circulation resuntling in thrombocytopenia and low vWF levels.