Type IIB von Willebrand disease: a paradox explains how von Willebrand factor works.
Type IIB is a variant form of von Willebrand disease in which a structural abnormality of von Willebrand factor ( VWF) causes enhanced binding to the platelet glycoprotein Ib receptor. As a consequence of this functional alteration, there is a decrease in the concentration of the largest VWF multimers in plasma, and the platelet count may be episodically decreased as a consequence of microaggregation. The net result is an apparent paradox, since the presence of a hyperfunctional adhesive molecule in blood causes a bleeding tendency. Here I recall how my colleagues and I managed to understand what goes on in these patients.[1]References
- Type IIB von Willebrand disease: a paradox explains how von Willebrand factor works. Ruggeri, Z.M. J. Thromb. Haemost. (2004) [Pubmed]
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