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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