Disease relevance of Purpura, Thrombotic Thrombocytopenic

• All 6 patients with familial thrombotic thrombocytopenic purpura lacked von Willebrand factor-cleaving protease activity but had no inhibitor, whereas all 10 patients with familial hemolytic-uremic syndrome had normal protease activity [1].
• Neurological manifestations of systemic lupus erythematosus and thrombotic thrombocytopenic purpura. [2].
• PMPs are observed in patients with acute myocardial infarction, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, heparin-induced thrombocytopenia and other thrombotic disorders, but the importance of circulating PMPs in the pathogenesis of these diseases is still debated [3].
• Protein C levels in disseminated intravascular coagulation and thrombotic thrombocytopenic purpura: its correlation with other coagulation parameters [4].
• Endothelin: a possible role in the occurrence of renal failure in thrombotic thrombocytopenic purpura [5].

High impact information on Purpura, Thrombotic Thrombocytopenic

• Because of the association between ticlopidine use and thrombotic thrombocytopenic purpura and other adverse effects, clopidogrel has largely replaced ticlopidine in clinical practice [6].
• Thrombotic thrombocytopenic purpura associated with clopidogrel [6].
• Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura [7].
• Incidence and clinical course of thrombotic thrombocytopenic purpura due to ticlopidine following coronary stenting. EPISTENT Investigators. Evaluation of Platelet IIb/IIIa Inhibitor for Stenting [8].
• Ineffective epoprostenol therapy for thrombotic thrombocytopenic purpura [9].

Chemical compound and disease context of Purpura, Thrombotic Thrombocytopenic

• Ticlopidine and thrombotic thrombocytopenic purpura [10].
• Thrombotic thrombocytopenic purpura: onset during dipyridamole therapy [11].
• Cyclophosphamide for chronic relapsing thrombotic thrombocytopenic purpura [12].
• Remission after 13-cis retinoic acid in thrombotic thrombocytopenic purpura [13].
• Nafazatrom in treatment of thrombotic thrombocytopenic purpura [14].

Biological context of Purpura, Thrombotic Thrombocytopenic

• The onset of ticlopidine-associated thrombotic thrombocytopenic purpura is difficult to predict, despite close monitoring of platelet counts [15].
• Studies investigating platelet aggregation and release initiated by sera from patients with thrombotic thrombocytopenic purpura [16].
• We report here 7 new mutations in the ADAMTS13 gene responsible for Upshaw-Schulman syndrome (USS), a catastrophic phenotype of congenital thrombotic thrombocytopenic purpura, by analyzing 5 Japanese families [17].
• Unlike cases of chronic relapsing thrombotic thrombocytopenic purpura, vWF-cleaving protease activity was present and treatment of cultured human endothelial cells with the patient's plasma did not induce apoptosis [18].
• Fibrinolysis in health and disease: abnormal levels of plasminogen activator, plasminogen activator inhibitor, and protein C in thrombotic thrombocytopenic purpura [19].

Anatomical context of Purpura, Thrombotic Thrombocytopenic

• Remission plasma samples of some patients with chronic relapsing thrombotic thrombocytopenic purpura (TTP) contain unusually large von Willebrand factor (vWF) multimers similar to those produced by normal human endothelial cells in culture [20].
• Protein C was measured by means of enzyme-linked immunosorbent assay (ELISA) in plasmas from 58 normal subjects, 39 patients with disseminated intravascular coagulation (DIC) and 5 patients with thrombotic thrombocytopenic purpura (TTP) [4].
• Cyclosporin for thrombotic thrombocytopenic purpura after autologous bone marrow transplantation [21].
• Thrombotic microangiopathy (TMA) that occurs after stem cell transplantation (SCT) is generally regarded as being different from thrombotic thrombocytopenic purpura (TTP), because it is reportedly not associated with deficiency of von Willebrand factor-cleaving protease, whereas this enzyme is deficient in TTP [22].
• Our group has previously reported elevations of EMP in patients with coronary artery disease (CAD), thrombotic thrombocytopenic purpura (TTP), pre-eclampsia, multiple sclerosis (MS), and severe hypertension (HTN) [23].

Gene context of Purpura, Thrombotic Thrombocytopenic

• ADAMTS13 deficiency is linked to a life-threatening disorder, thrombotic thrombocytopenic purpura (TTP), characterized by platelet-rich thrombi in the microvasculature [24].
• Injection of human TSP-1 or thrombotic thrombocytopenic purpura (TTP) patient-derived neutralizing anti-ADAMTS13 antibodies corrected the defective platelet recruitment in Tsp1-/- mice, while having a moderate effect in WT mice [25].
• Increased plasma thrombomodulin as a vascular endothelial cell marker in patients with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome [26].
• Conversely, defects in the feedback proteolysis of VWF by ADAMTS-13 can cause thrombotic thrombocytopenic purpura (TTP) [27].
• Platelet-agglutinating protein p37 from a thrombotic thrombocytopenic purpura plasma forms complexes with platelet membrane glycoprotein IV (CD36) [28].

Analytical, diagnostic and therapeutic context of Purpura, Thrombotic Thrombocytopenic

• Seven of eight patients with thrombotic thrombocytopenic purpura who were treated with both exchange plasmapheresis and antiplatelet agents (aspirin and dipyridamole) achieved complete remission [29].
• A 43-year-old woman who developed thrombotic thrombocytopenic purpura was treated successfully with high doses of prednisone and a splenectomy [30].
• Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura [31].
• Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura [32].
• An enzyme immunoassay of ADAMTS13 distinguishes patients with thrombotic thrombocytopenic purpura from normal individuals and carriers of ADAMTS13 mutations [33].

References