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| | eMedicine - Von Willebrand Disease : Article by John D Geil, MD (Site not responding. Last check: 2007-10-07) |
 | | Background: Although referred to as a single disease, von Willebrand disease (vWD) is in fact a family of bleeding disorders caused by an abnormality of the von Willebrand factor (vWF). |
 | | Pathophysiology: vWD is due to an abnormality, either quantitative or qualitative, of the vWF, which is a large multimeric glycoprotein that functions as the carrier protein for factor VIII (FVIII). |
 | | In the homozygous patient, type 3 vWD is characterized by marked deficiencies of both vWF and FVIIIc in the plasma, the absence of vWF from both platelets and endothelial cells, and a lack of the secondary transfusion response and the response to DDAVP. |
| www.emedicine.com /ped/topic2419.htm (3411 words) |
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