title: ADAMTS-13 assays in thrombotic thrombocytopenic purpura.
abstract: ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but life threatening disease characterized by microangiopathic haemolytic anaemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis and variable signs and symptoms of organ ischemia and damage. In the last few years, a number of in house and commercial laboratory assays for ADAMTS-13 and its autoantibodies have been developed. The features and clinical utility of ADAMTS-13 assays are summarized in this review.
authors.affiliation: Department of Medicine and Medical Specialities, Angelo Bianchi Bonomi Hemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Università degli Studi di Milano, Luigi Villa Foundation, Milan, Italy. flora.peyvandi@unimi.it
authors.forename: F
authors.initials: F
authors.lastname: Peyvandi
authors.forename: R
authors.initials: R
authors.lastname: Palla
authors.forename: L A
authors.initials: LA
authors.lastname: Lotta
authors.forename: I
authors.initials: I
authors.lastname: Mackie
authors.forename: M A
authors.initials: MA
authors.lastname: Scully
authors.forename: S J
authors.initials: SJ
authors.lastname: Machin
mesh_terms: D051722:ADAM Proteins / Q000097:blood* / Q000172:deficiency / Q000235:genetics / Q000276:immunology; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D004796:Clinical Enzyme Tests / Q000592:standards; D003937:Diagnosis, Differential; D006801:Humans; D007118:Immunoassay / Q000592:standards; D011237:Predictive Value of Tests; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics / Q000276:immunology; D011933:Reagent Kits, Diagnostic
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D011933:Reagent Kits, Diagnostic; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human