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von Willebrand disease (VWD): evidence-based diagnosis and
management guidelines, the National Heart, Lung, and Blood
Institute (NHLBI) Expert Panel report (USA)1
Summary. von Willebrand disease (VWD) is a commonly
encountered inherited bleeding disorder
affecting both males and females, causing mucous
membrane and skin bleeding symptoms, and bleeding
with surgical or other haemostatic challenges.
VWD may be disproportionately symptomatic in
women of child-bearing age. It may also occur less
frequently as an acquired disorder (acquired von
Willebrand syndrome). VWD is caused by deficiency
or dysfunction of von Willebrand factor (VWF),
a plasma protein that mediates platelet haemostatic
function and stabilizes blood coagulation factor VIII.
The pathophysiology, classification, diagnosis and
management of VWD are relatively complex, but
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