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Question 2

A 32 year old man is referred to haematology from A&E following a severe episode of epistaxis which was arrested by local measures.  He required transfusion of 2 units of blood.  He has a history of recurrent epistaxis and had excessive bleeding following tonsillectomy aged 6.  Initial investigations were as follows:

Hb 8.6g/dL, WCC 16.4, Plts 480
vWF Ag 30u/dL, vWF Ac (rico) 10u/dL
FVIII 100u/dL
Collagen binding studies normal
RIPA reduced
vWF multimers normal

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