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Prepublished online as a Blood First Edition Paper on July 10, 2003; DOI 10.1182/blood-2003-05-1616. This Article Full Text (PDF) All Versions of this Article: 2003-05-1616v1 102/9/3241    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Scheiflinger, F. Articles by Rieger, M. Search for Related Content PubMed PubMed Citation Articles by Scheiflinger, F. Articles by Rieger, M. Social Bookmarking                   What's this? Submitted May 22, 2003 Accepted June 30, 2003 Non-neutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura (TTP) Friedrich Scheiflinger*, Paul Knoebl, Bettina Trattner, Barbara Plaimauer, Gabriele Mohr, Michael Dockal, Friedrich Dorner, and Manfred Rieger Biomedical Research Center, Baxter BioScience, Orth/Donau, Austria Department of Medicine, Division of Hematology and Blood Coagulation, University of Vienna, Vienna, Austria * Corresponding author; email: scheiff{at}baxter.com. Acute thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by inhibiting autoantibodies to ADAMTS-13. We report the case of a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely used fluid phase activity assay. With a newly developed ELISA, using immobilized recombinant ADAMTS-13, we found high titers of IgM and IgG antibodies that bound to ADAMTS-13, but did not neutralize the protease activity. These autoantibodies probably influenced the half-life of ADAMTS-13 and/or its binding to the endothelial cell surface, thereby compromising ADAMTS-13 activity in vivo. Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution and the epitope mapping of non-neutralizing antibodies will be an important area for future research. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: B. Lammle, J. A. Kremer Hovinga, and J. N. George Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease Haematologica, February 1, 2008; 93(2): 172 - 177. [Full Text] [PDF] S. Ferrari, F. Scheiflinger, M. Rieger, G. Mudde, M. Wolf, P. Coppo, J.-P. Girma, E. Azoulay, C. Brun-Buisson, F. Fakhouri, et al. Prognostic value of anti-ADAMTS13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS13 activity Blood, April 1, 2007; 109(7): 2815 - 2822. [Abstract] [Full Text] [PDF] P. M. Mannucci and F. Peyvandi TTP and ADAMTS13: When Is Testing Appropriate? Hematology, January 1, 2007; 2007(1): 121 - 126. [Abstract] [Full Text] [PDF] A. K. Chauhan, D. G. Motto, C. B. Lamb, W. Bergmeier, M. Dockal, B. Plaimauer, F. Scheiflinger, D. Ginsburg, and D. D. Wagner Systemic antithrombotic effects of ADAMTS13 J. Exp. Med., March 20, 2006; 203(3): 767 - 776. [Abstract] [Full Text] [PDF] J. E. Sadler Thrombotic Thrombocytopenic Purpura: A Moving Target Hematology, January 1, 2006; 2006(1): 415 - 420. [Abstract] [Full Text] [PDF] F. Fakhouri, J.-P. Vernant, A. Veyradier, M. Wolf, G. 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