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 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 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 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 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 Biomarker disease BEFREE Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura. 27479501 2016
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 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 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 Antibodies anti-ADAMTS13 were positive, establishing the diagnosis of acquired thrombotic thrombocytopenic purpura. 29550758 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE We anticipate that our findings may be relevant for the initiation of autoimmune reactivity against ADAMTS13 in patients with acquired TTP. 24977290 2014
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression disease BEFREE Diagnosis of aTTP was confirmed if ADAMTS13 level were < 10% and/or the medical records explicitly mentioned aTTP diagnosis. 31730475 2019
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 Click to hear Dr Cataland's presentation on acquired thrombotic thrombocytopenic purpura SUMMARY: Background Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor-cleaving protease ADAMTS-13. 27762046 2016
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 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 AlteredExpression disease BEFREE Congenital TTP is caused by a defect in the ADAMTS13 gene resulting in decreased or absent enzyme activity; acquired TTP results from autoantibodies that either inhibit the activity or increase the clearance of ADAMTS13. 26581428 2016
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 AlteredExpression disease BEFREE ADAMTS13 activity <10% defined those with idiopathic/acquired TTP (41/70). 31778944 2020
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 Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. 29564686 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Modifying ADAMTS13 to modulate binding of pathogenic autoantibodies of patients with acquired thrombotic thrombocytopenic purpura. 31753929 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE However, undetectably low plasma ADAMTS13 activity in the pre-treatment sample, associated with inhibitory ADAMTS13 antibodies, subsequently changed the diagnosis to acquired TTP. vWF protease activity normalized within 15 months without further treatment, and the patient remained in long-term clinical and laboratory remission. 29728803 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression disease BEFREE ADAMTS13 activity is used for confirmation of the diagnosis of acquired TTP (97%). 29150875 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE The results from this study provide a basis for the identification of immuno-dominant epitopes on ADAMTS13 involved in the onset of acquired thrombotic thrombocytopenic purpura. 29567779 2018