title: Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship.
abstract: We present a patient with a history of benign monoclonal gammopathy, who developed thrombotic thrombocytopenic purpura (TTP), initially presenting as bilateral serous retinal detachment. Plasma of the patient contained high titers of anti ADAMTS13 antibodies that were directed towards the disintegrin/TSR1/cysteine-rich/spacer and CUB1-2 domains. ADAMTS13 activity was undetectable. Total IgG purified from plasma of the patient partially inhibited ADAMTS13 activity. In contrast, the isolated M-protein did neither bind to, nor inhibit activity of ADAMTS13. We conclude that in this patient the monoclonal gammopathy and TTP co-existed as distinct pathological entities.
authors.affiliation: Department of Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. N.Riksen@aig.umcn.nl
authors.forename: Niels P
authors.initials: NP
authors.lastname: Riksen
authors.forename: Brenda M
authors.initials: BM
authors.lastname: Luken
authors.forename: Ina S
authors.initials: IS
authors.lastname: Klasen
authors.forename: Jan
authors.initials: J
authors.lastname: Voorberg
authors.forename: Niels
authors.initials: N
authors.lastname: Crama
authors.forename: Marcel
authors.initials: M
authors.lastname: van Deuren
mesh_terms: D051722:ADAM Proteins / Q000276:immunology*; D000071120:ADAMTS13 Protein; D048868:Adaptor Proteins, Signal Transducing; D000328:Adult; D000911:Antibodies, Monoclonal / Q000096:biosynthesis* / Q000097:blood; D015703:Antigens, CD / Q000276:immunology*; D000951:Antigens, Neoplasm; D002135:Calcium-Binding Proteins; D015815:Cell Adhesion Molecules / Q000276:immunology*; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000096:biosynthesis / Q000097:blood; D008565:Membrane Proteins / Q000276:immunology*; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis / Q000235:genetics / Q000276:immunology*; D009363:Neoplasm Proteins / Q000276:immunology*; D017434:Protein Structure, Tertiary
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D000911:Antibodies, Monoclonal; D015703:Antigens, CD; D000951:Antigens, Neoplasm; C432044:CDCP1 protein, human; D002135:Calcium-Binding Proteins; D015815:Cell Adhesion Molecules; D007075:Immunoglobulin M; D008565:Membrane Proteins; D009363:Neoplasm Proteins; C469999:SCUBE2 protein, human; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human