von Willebrand factor and thrombotic thrombocytopenic purpura. Curr Opin Hematol 2000 Sep;7(5):278-83
Date
08/29/2000Pubmed ID
10961577DOI
10.1097/00062752-200009000-00004Scopus ID
2-s2.0-0033883648 (requires institutional sign-in at Scopus site) 11 CitationsAbstract
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.
Author List
Raife TJ, Montgomery RRAuthor
Robert R. Montgomery MD Emeritus Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
ADAM ProteinsADAMTS13 Protein
Animals
Humans
Metalloendopeptidases
Microcirculation
Purpura, Thrombotic Thrombocytopenic
Thrombosis
von Willebrand Factor
