title: Evolution of ADAMTS13 antibodies in a fatal case of thrombotic thrombocytopenic purpura.
abstract: In a patient with fatal thrombotic thrombocytopenic purpura, the inhibitory activity of antibodies against ADAMTS13 rapidly escalated to extremely high levels despite daily plasma exchange and corticosteroid therapy. This increase was found to be because of a combination of higher antibody concentration and potency. Furthermore, during her course of the disease, the percentage of IgG(1) antibody progressively decreased whereas that of IgG(2) antibody increased, suggesting Th1-type cytokine response. These changes suggest that the course of TTP may be exacerbated by complex immune reactions. Further characterization of the factors contributing to this exacerbation may have important pathogenetic and therapeutic implications.
authors.affiliation: Unified Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, Bronx, New York 10467, USA.
authors.forename: Lingli
authors.initials: L
authors.lastname: Dong
authors.forename: Visalam
authors.initials: V
authors.lastname: Chandrasekaran
authors.forename: Wenhua
authors.initials: W
authors.lastname: Zhou
authors.forename: Han-Mou
authors.initials: HM
authors.lastname: Tsai
mesh_terms: D051722:ADAM Proteins / Q000097:blood* / Q000235:genetics / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D004791:Enzyme Inhibitors / Q000097:blood; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000145:classification / Q000276:immunology* / Q000302:isolation & purification; D010951:Plasma Exchange / Q000009:adverse effects / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000276:immunology* / Q000628:therapy; D011994:Recombinant Proteins / Q000097:blood / Q000378:metabolism
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D004791:Enzyme Inhibitors; D007074:Immunoglobulin G; D011994:Recombinant Proteins; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human