VWF, von Willebrand factor, 7450

N. diseases: 498; N. variants: 158
Disease: Thrombotic thrombocytopenic purpura, acquired ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Recently, caplacizumab, a bivalent nanobody targeting the glycoprotein 1b binding epitope of von Willebrand factor A1 domain, was approved as an addition to the current regimen of plasma exchange and immunomodulation for adult patients of acquired thrombotic thrombocytopenic purpura. 30928346 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Loss of von Willebrand factor high-molecular-weight multimers at acute phase is associated with detectable anti-ADAMTS13 IgG and neurological symptoms in acquired thrombotic thrombocytopenic purpura. 31330376 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes, and tissue injury that characterize TTP. 28768626 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Acquired thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease in which ultralarge von Willebrand factor (UL-VWF) multimers accumulate as a result of autoantibody inhibition of the VWF protease, ADAMTS13. 27040023 2016
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE A functional deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif), a von-Willebrand factor (VWF) cleaving protease, is central to the pathogenesis of congenital and acquired thrombotic thrombocytopenic purpura (TTP). 23420593 2013
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 AlteredExpression disease BEFREE These results suggest that VWF immobilization and high shear flow yielded optimal conditions for ADAMTS-13 activity, and that introduction of BaCl2 in the Impact-R (CPA) test may be useful for differentiation between inherited and acquired TTP. 16894459 2006
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE In particular, the cysteine-rich/spacer domains are essential for VWF cleavage and are the principal epitopes recognized by autoantibodies in patients with acquired TTP. 15662617 2005
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Severe deficiency of von Willebrand factor (VWF)-cleaving protease (ADAMTS-13) activity (<5% of normal) is a specific finding for acute idiopathic thrombotic thrombocytopenic purpura (TTP), a disorder that presents as thrombocytopenia, microangiopathic hemolytic anemia, and often organ dysfunction such as neurological disturbances or renal failure, and fever. 14727262 2004
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.090 Biomarker disease BEFREE Furthermore, recombinant ADAMTS13-mediated degradation of VWF multimers was entirely inhibited in the presence of plasma from a patient with acquired TTP. 12393399 2002