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Blood, 1 November 2003, Vol. 102, No. 9, pp. 3241-3243. Prepublished online as a Blood First Edition Paper on July 10, 2003; DOI 10.1182/blood-2003-05-1616. This Article Full Text 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 Citation Map 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. Related Collections Hemostasis, Thrombosis, and Vascular Biology Cell Adhesion and Motility Brief Reports Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Brief report Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura Friedrich Scheiflinger, Paul Knöbl, Bettina Trattner, Barbara Plaimauer, Gabriele Mohr, Michael Dockal, Friedrich Dorner, and Manfred Rieger From Baxter BioScience, Biomedical Research Center, 2304 Orth/Donau, Austria; and Department of Medicine 1, Division of Hematology and Blood Coagulation, University of Vienna, Austria. Acquired thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by autoantibodies inhibitory to ADAMTS-13. We report data on 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 enzyme-linked immunosorbent assay, using immobilized recombinant ADAMTS-13, we found high titers of IgM and IgG antibodies that bound to ADAMTS-13, but did not neutralize protease activity. These autoantibodies probably influenced the half-life of ADAMTS-13 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 nonneutralizing 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. Kaplanski, R. Binaut, M. Rieger, F. Scheiflinger, P. Poullin, B. Deroure, et al. Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases Blood, September 15, 2005; 106(6): 1932 - 1937. [Abstract] [Full Text] [PDF] M. Rieger, P. M. Mannucci, J. A. K. Hovinga, A. Herzog, G. Gerstenbauer, C. Konetschny, K. Zimmermann, I. Scharrer, F. Peyvandi, M. Galbusera, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases Blood, August 15, 2005; 106(4): 1262 - 1267. [Abstract] [Full Text] [PDF] M. Galbusera, E. Bresin, M. Noris, S. Gastoldi, D. Belotti, C. Capoferri, E. Daina, P. Perseghin, F. Scheiflinger, F. Fakhouri, et al. Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report Blood, August 1, 2005; 106(3): 925 - 928. [Abstract] [Full Text] [PDF] G. G. Levy, D. G. Motto, and D. Ginsburg ADAMTS13 turns 3 Blood, July 1, 2005; 106(1): 11 - 17. [Abstract] [Full Text] [PDF] M. Noris, S. Bucchioni, M. Galbusera, R. Donadelli, E. Bresin, F. Castelletti, J. Caprioli, S. Brioschi, F. Scheiflinger, G. Remuzzi, et al. Complement Factor H Mutation in Familial Thrombotic Thrombocytopenic Purpura with ADAMTS13 Deficiency and Renal Involvement J. Am. Soc. Nephrol., May 1, 2005; 16(5): 1177 - 1183. [Abstract] [Full Text] [PDF] M.-A. Dragon-Durey, C. Loirat, S. Cloarec, M.-A. Macher, J. Blouin, H. Nivet, L. Weiss, W. H. Fridman, and V. Fremeaux-Bacchi Anti-Factor H Autoantibodies Associated with Atypical Hemolytic Uremic Syndrome J. Am. Soc. Nephrol., February 1, 2005; 16(2): 555 - 563. [Abstract] [Full Text] [PDF] J.-D. Studt, J. A. K. Hovinga, G. Antoine, M. Hermann, M. Rieger, F. Scheiflinger, and B. Lammle Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin Blood, January 15, 2005; 105(2): 542 - 544. [Abstract] [Full Text] [PDF] G. Ferretti, M. C. Petti, P. Carlini, M. Zeuli, A. Picardi, G. Meloni, E. Bria, P. Papaldo, A. Fabi, and F. Cognetti Zoledronic acid-associated thrombotic thrombocytopenic purpura Ann. Onc., December 1, 2004; 15(12): 1847 - 1848. [Full Text] [PDF] C. Klaus, B. Plaimauer, J.-D. Studt, F. Dorner, B. Lammle, P. M. Mannucci, and F. Scheiflinger Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura Blood, June 15, 2004; 103(12): 4514 - 4519. [Abstract] [Full Text] [PDF] X. L. Zheng, R. M. Kaufman, L. T. Goodnough, and J. E. Sadler Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura Blood, June 1, 2004; 103(11): 4043 - 4049. [Abstract] [Full Text] [PDF] J.-D. Studt, J. A. K. Hovinga, R. Radonic, V. Gasparovic, D. Ivanovic, M. Merkler, U. Wirthmueller, C. Dahinden, M. Furlan, and B. Lammle Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins Blood, June 1, 2004; 103(11): 4195 - 4197. [Abstract] [Full Text] [PDF]

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