von Willebrand factor and thrombotic thrombocytopenic purpura.
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 thrombotic 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 thrombotic 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 thrombotic microangiopathy patients and might be a rational basis for future classification of thrombotic microangiopathic disorders.
Raife
T J
TJ
The Blood Research Institute of The Blood Center of Southeastern Wisconsin, The Medical College of Wisconsin, Milwaukee 53201-2178, USA.
Montgomery
R R
RR
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000818:Animals; D006801:Humans; D008666:Metalloendopeptidases / Q000097:blood / Q000172:deficiency / Q000502:physiology; D008833:Microcirculation / Q000378:metabolism / Q000503:physiopathology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000209:etiology / Q000503:physiopathology*; D013927:Thrombosis / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D014841:von Willebrand Factor / Q000009:adverse effects / Q000378:metabolism / Q000502:physiology*
D014841:von Willebrand Factor; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human