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 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 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 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 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 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 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 ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 2. Pathogenicity in an animal model. 27040023 2016
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 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 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 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 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 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 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 Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy. 27720178 2016
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 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 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
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 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 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