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 		0.100 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
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE There is also an enduring uncertainty about the specificity of ADAMTS13 deficiency for the diagnosis of acquired TTP and a perception that the result does not alter patient management. 12656756 2003
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression 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 		0.100 Biomarker disease BEFREE In vitro expression and characterization of recombinant ADAMTS-13 (rADAMTS-13) clearly established that ADAMTS-13 is deficient in congenital TTP and inhibited in acquired TTP. 14727256 2004
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE Autoantibodies that inactivate ADAMTS13 are the most frequent cause of acquired TTP. 14976043 2004
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE We report identical twin sisters suffering from acquired TTP due to immunoglobulin G (IgG) autoantibodies inactivating ADAMTS13, suggesting an important role of hitherto unidentified genetic determinants of ADAMTS13 inhibitor formation. 14982879 2004
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression disease BEFREE Mutations in the ADAMTS13 gene cause inherited TTP, and the development of autoantibodies that inhibit ADAMTS13 activity frequently are associated with acquired TTP. 15662617 2005
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Acquired TTP is often but not always associated with severe, autoantibody-mediated ADAMTS-13 deficiency. 16102032 2005
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Plasma of six patients with acquired TTP competed for binding of scFv I-9 to ADAMTS13. 16898953 2006
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Unlike acquired thrombotic thrombocytopenic purpura or haemolytic uraemic syndrome, which are often intractable, thrombotic microangiopathy in patients with Upshaw-Schulman syndrome (USS)--a congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS13) activity--responds very well to plasma infusion and does not even require plasma exchange. 16449289 2006
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression disease BEFREE We applied the Impact-R system [Cone and plate(let) Analyzer, CPA] to determine optimal conditions for ADAMTS-13 function, to assess it's activity in TTP patients and to distinguish inherited TTP (inTTP) from acquired TTP (acTTP). 16894459 2006
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE ADAMTS13 was not detected in the plasma from patients with congenital TTP (n = 5) by either antibody, whereas patients with acquired TTP (n = 2) presented the normal phenotype. 17187257 2007
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE These results suggest that VH1-69 derived antibodies directed towards ADAMTS13 develop in the majority of patients with acquired TTP. 19054323 2009
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Congenital TTP occurs due to ADAMTS13 mutations, with the usual debut occurring during the first years of life, while acquired TTP is associated with auto-antibodies against ADAMTS13. 18807073 2009
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE We now show that replacement of R660, Y661, or Y665 with alanine in ADAMTS13 reduced/abolished the binding of 2 previously isolated human monoclonal antibodies and polyclonal antibodies derived from plasma of 6 patients with acquired TTP. 20032502 2010
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE Deficiency of ADAMTS13, due to autoimmune inhibitors in patients with acquired TTP and mutations of the ADAMTS13 gene in hereditary cases, leads to VWF-platelet aggregation and microvascular thrombosis of TTP. 20058209 2010
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Finally, a model is presented for the development of anti-ADAMTS13 antibodies in previously healthy individuals that incorporates the recent identification of HLA DRB1*11 as a risk factor for acquired TTP. 21535387 2011
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type-1 motifs 13) has been shown to be of major pathophysiological importance for thrombotic microangiopathy (TMA) in the setting of thrombocytic thrombocytopenic purpura (TTP) when either lacking (inherited TTP) or if antibodies against ADAMTS13 are present (acquired TTP). 21531732 2011
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Together, our findings suggest that the novel cell-based assay may be applicable for rapid identification and mapping of anti-ADAMTS13 autoantibodies in patients with acquired TTP. 21901237 2011
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE The autoantibody-resistant ADAMTS13 variants may be further developed as a novel therapeutic for acquired TTP with inhibitors. 22289888 2012
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE Genetic mutations in and acquired autoantibodies to ADAMTS13 cause congenital TTP (termed Upshaw-Schulman syndrome [USS]) and acquired TTP, respectively. 22264931 2012
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Recombinant ADAMTS13 ought to be soon available for congenital TTP, while acquired TTP children might benefit from its administration, alone or in association with rituximab, to avoid or limit plasma exchange duration. 23422353 2013
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 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 		0.100 Biomarker disease BEFREE ADAMTS13 activity evaluation and detection of anti-ADAMTS13 antibody could help to predict the risk of complications in pregnant women with a history of acquired TTP. 25431165 2014
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE We also present data on 12 women with a history of nonpregnancy-associated TTP: 18 subsequent pregnancies have been successfully managed, guided by ADAMTS13 levels. cTTP presents more frequently than acquired TTP during pregnancy and must be differentiated by ADAMTS13 analysis. 24859360 2014