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 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 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 		0.100 Biomarker disease BEFREE In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. 30625070 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by the immune-mediated severe deficiency of ADAMTS13. 30861548 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE In patients treated for acquired thrombotic thrombocytopenic purpura (TTP), a persistently severe ADAMTS13 deficiency (<10%) in remission is associated with more relapses. 30471507 2019
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 AlteredExpression disease BEFREE Objectives To report our experience of management of six patients with acquired TTP who underwent elective surgery after prophylactic treatment to restore ADAMTS-13 activity levels. 30629316 2019
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 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 Next, the conformation of ADAMTS-13 in 40 healthy donors (HDs), 99 aTTP patients (63 in the acute phase versus 36 in remission), 12 hemolytic-uremic syndrome (HUS) patients and 63 sepsis patients was determined with ELISA. 29222940 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Concentration and Subclass Distribution of Anti-ADAMTS13 IgG Autoantibodies in Different Stages of Acquired Idiopathic Thrombotic Thrombocytopenic Purpura. 30061898 2018
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 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 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
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange. 30178615 2018
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE We report two cases of acquired TTP in Caucasian sisters with inactive ADAMTS13 metalloprotease due to ADAMTS13 autoantibodies suggesting a role of genetic determinants in this life-threatening disease. 27859681 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE ADAMTS13 test and/or PLASMIC clinical score in management of acquired thrombotic thrombocytopenic purpura: a cost-effective analysis. 28646526 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE Although plasma infusion is often sufficient for prophylaxis or treatment of hereditary TTP due to ADAMTS-13 mutations, daily therapeutic plasma exchange remains the initial treatment of choice for acquired TTP with demonstrable autoantibodies. 28662310 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Acquired thrombotic thrombocytopenic purpura is a rare and severe disease characterized by auto-antibodies directed against "A Disintegrin And Metalloproteinase with Thrombospondin type 1 repeats, 13<sup>th</sup> member" (ADAMTS13), a plasma protein involved in hemostasis. 28751567 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE We measured ADAMTS13-specific CICs in 51 patients with severe ADAMTS13 deficiency and anti-ADAMTS13 autoantibodies, at the first episode of acquired TTP. 27887777 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Plasma exchange therapy using FFP is conducted in patients with acquired TTP to supplement ADAMTS13 and remove anti-ADAMTS13 autoantibodies. 28550351 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 GeneticVariation disease BEFREE The acquired form of idiopathic thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease, in which the underlying ADAMTS13-deficiency is caused by inhibitory autoantibodies against the protease. 27866840 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). 28759473 2017
CUI: C2584778
Disease: Thrombotic thrombocytopenic purpura, acquired
Thrombotic thrombocytopenic purpura, acquired 		0.100 Biomarker disease BEFREE Methods Twenty-five patients with acute aTTP and/or severe a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency were admitted to our centre from April 2009 to March 2015. 28639502 2017