The Unrecognized von Willebrand Disease: A Frequent Cause of Bleeding Complications

Quantitative and qualitative defects of von Willebrand factor (VWF) cause von Willebrand disease (VWD), the most common inborn bleeding disorder being inherited in a mainly autosomal dominant but also recessive manner. According to its modular structure characterized by distinct functional and structural domains, VWF defects correlate with considerable heterogeneity of clinical symptoms, biochemical parameters and of the underlying molecular mechanisms. This renders the diagnostic approach demanding and is also the reason for under- or misdiagnosis of VWD. (C) 2008 S. Karger GmbH, Freiburg i.Br.