Disease: Purpura, Thrombotic Thrombocytopenic ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE The vast majority of cases of thrombotic thrombocytopenic purpura (TTP) are the result of acquired antibodies which inhibit the activity of the ADAMTS13 enzyme. 31167181 2020
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE Seventy out of 71 patients with suspected TTP who had ADAMTS13 activity measured were included. 31778944 2020
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Thrombotic thrombocytopenic purpura (TTP) is a rare life threatening thrombotic microangiopathy caused by a severe functional deficiency of ADAMTS13, most frequently due to autoantibodies to ADAMTS13, thus termed acquired autoimmune TTP. 31330376 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE For example, HLA-DRB1 *11 presents the self-antigen and induces an immune response against ADAMTS13, which is associated with thrombocytopenia in TTP patients. 30543580 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Recombinant ADAMTS13 is also under development to replace plasma infusion as the therapy for hereditary thrombotic thrombocytopenic purpura. 30928346 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. 31585956 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Positive Response and Increase in ADAMTS13 with Scheduled Rituximab in a Patient with Relapsing Thrombotic Thrombocytopenic Purpura. 31516768 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission. 31431443 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE Comparison of ADAMTS13 and Von Willebrand factor levels and activities, and plasminogen levels, in plasma products currently available for the treatment of thrombotic thrombocytopenic purpura in South Africa. 30551950 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 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: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE Severe deficiency of plasma ADAMTS13 activity is the primary cause of thrombotic thrombocytopenic purpura (TTP) whereas overwhelming activation of complement via an alternative pathway results in atypical hemolytic uremic syndrome (aHUS), the prototypes of thrombotic microangiopathy (TMA). 31409673 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE Because of its short turnaround time and full automation, the HemosIL AcuStar ADAMTS13 activity assay might become the assay of choice to rapidly test ADAMTS13 activity in plasma and thus establish the diagnosis of acute TTP in emergency settings. 31587247 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 GeneticVariation disease BEFREE In this Chinese cohort, 17 patients were diagnosed with TTP according to ADAMTS13 activity results. 31724781 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE The discovery of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) and its utility in the diagnosis of thrombotic thrombocytopenic purpura (TTP) has resulted in the appreciation that cases of aHUS have been inappropriately diagnosed as TTP. 31198222 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Histone-induced thrombotic thrombocytopenic purpura in adamts13-/- zebrafish depends on von Willebrand factor. 31753928 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 GeneticVariation disease BEFREE Inherited TTP or USS is a rare autosomal recessive inherited disease leading to a severe deficiency of ADAMTS13 mostly beginning in childhood or in young female adult during pregnancy. 30930238 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 GeneticVariation disease BEFREE The best described abnormality is the congenital form of thrombotic thrombocytopenic purpura (TTP) resulting from germline mutations in the ADAMTS-13 gene. 30593735 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 AlteredExpression disease BEFREE Unique case of autoantibody mediated inactivation of ADAMTS13 in an Indian TTP patient. 30953939 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Thrombotic Thrombocytopenic Purpura (TTP) is a rare life-threatening disease caused by ADAMTS-13 deficiency. 30917902 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE ADAMTS13 conformation could be a promising parameter for TTP diagnosis and prognosis in the coming years. 31107120 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Several conditions, including surgery, may influence the levels of ultra-large von Willebrand factor and ADAMTS-13, acting as a trigger for an acute TTP event. 30629316 2019
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 GeneticVariation disease BEFREE In contrast, disseminated intravascular microthrombosis (DIT) occurs in thrombotic thrombocytopenic purpura (TTP) and TTP-like syndrome due to ADAMTS13 deficiency or insufficiency. 29621007 2018
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Efficacy of eculizumab in severe ADAMTS13-deficient thrombotic thrombocytopenic purpura (TTP) refractory to standard therapies. 29567368 2018
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 Biomarker disease BEFREE Force-regulated cleavage of A2 domain of von Willebrand factor (vWF) by ADAMTS13 is a key event in preventing thrombotic thrombocytopenic purpura (TTP). 29636514 2018
CUI: C0034155
Disease: Purpura, Thrombotic Thrombocytopenic
Purpura, Thrombotic Thrombocytopenic 		0.700 GeneticVariation disease BEFREE Hence, we hypothesized that activation of plasminogen to plasmin becomes a new-onset factor for TTP due to ADAMTS13 inactivation. 29228282 2018