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Von Willebrand factor and thrombotic thrombocytopenic purpura

Current Opinion in Hematology, ISSN: 1065-6251, Vol: 7, Issue: 5, Page: 278-283
2000
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Review Description

Recent advances in the understanding of platelet-dependent hemostasis and von Willebrand factor (vWF) functional regulation offer new insights into the pathogenesis of thrombotic microangiopathic disorders. The discovery of vWF-cleaving protease activity in normal plasma, and its deficiency in thromboric thrombocytopenic purpura (TTP) patients, provides additional support for a pathologic role of ultra-large vWF in TTP. Although vWF-cleaving protease deficiency is highly prevalent among TTP patients, the defect has also been detected in individuals without active TTP. Therefore, vWF-cleaving protease deficiency appears to be an important risk factor for thromboric microangiopathy rather than a specific diagnostic marker of TTP. Recent data indicate that vWF-cleaving protease activity correlates with clinical parameters in thrombotic microangiopathy patients. Therefore, determination of vWF-cleaving protease activity might prove useful in the future care of thromboric microangiopathy patients and might be a rational basis for future classification of thrombotic microangiopathic disorders. (C) 2000 Lippincott Williams and Wilkins, Inc.

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