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Blood, 15 August 2005, Vol. 106, No. 4, pp. 1262-1267. Prepublished online as a Blood First Edition Paper on May 17, 2005; DOI 10.1182/blood-2004-11-4490. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-11-4490v1 2004-11-4490v2 106/4/1262    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 Rieger, M. Articles by Scheiflinger, F. Search for Related Content PubMed PubMed Citation Articles by Rieger, M. Articles by Scheiflinger, F. Related Collections Hemostasis, Thrombosis, and Vascular Biology Immunobiology Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases Manfred Rieger, Pier Mannuccio Mannucci, Johanna A. Kremer Hovinga, Andrea Herzog, Gabi Gerstenbauer, Christian Konetschny, Klaus Zimmermann, Inge Scharrer, Flora Peyvandi, Miriam Galbusera, Giuseppe Remuzzi, Martina Böhm, Barbara Plaimauer, Bernhard Lämmle, and Friedrich Scheiflinger From Baxter BioScience, Biomedical Research Center, Orth, Austria; the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Department of Internal Medicine and Dermatology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Maggiore Hospital and University of Milan, Milan, Italy; the Department of Hematology and Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland; the Mario Negri Institute for Pharmacological Research, Bergamo, Italy; the Department of Hemostaseology, University Hospital, Frankfurt, Germany; and the Division of Nephrology and Dialysis, Ospedali Riuniti di Bergamo, Italy. Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the protease and of acquired thrombotic thrombocytopenic purpura (TTP). We evaluated prevalence of anti-ADAMTS13 antibodies in 59 patients with thrombotic microangiopathies (TMAs) and in 160 patients with immunologic or thrombocytopenic diseases different from TTP, using an enzyme-linked immunosorbent assay (ELISA). Immunoglobulin G (IgG) antibodies directed against ADAMTS13 were found in 97% of untreated patients with acute acquired TMA who had plasma levels of ADAMTS13 activity below 10%. The corresponding prevalence of IgM antibodies was 11%. In contrast, anti-ADAMTS13 antibodies of G or M isotypes were detected in 20% of patients with TMA with ADAMTS13 activity above 10%. The ELISA was more sensitive than the standard functional inhibitor assay for detecting antibodies against ADAMTS13. Patients with thrombocytopenia from various causes (n = 50), systemic lupus erythematosus (SLE; n = 40), and the antiphospholipid antibody syndrome (APS; n = 55) had prevalences of IgG antibodies of 8%, 13%, and 5% respectively, only slightly higher than the prevalence in 111 healthy donors (4%). A rather high prevalence of anti-ADAMTS13 IgM antibodies was found in patients with SLE and APS (18% each). The clinical significance of IgM antibodies in these groups is unclear. In conclusion, the ELISA method detected anti-ADAMTS13 IgG antibodies in a very large proportion of patients with acquired TMA associated with severe ADAMTS13 deficiency, and was more sensitive than the inhibitor assay. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Peyvandi, S. Lavoretano, R. Palla, H. B. Feys, K. Vanhoorelbeke, T. Battaglioli, C. Valsecchi, M. T. Canciani, F. Fabris, S. Zver, et al. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission Haematologica, February 1, 2008; 93(2): 232 - 239. [Abstract] [Full Text] [PDF] N. P Riksen, B. M Luken, I. S Klasen, J. Voorberg, N. Crama, and M. van Deuren Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship Haematologica, June 1, 2007; 92(6): e74 - e76. [Abstract] [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] H. Izzedine, C. Isnard-Bagnis, V. Launay-Vacher, L. Mercadal, I. Tostivint, O. Rixe, I. Brocheriou, E. Bourry, S. Karie, S. Saeb, et al. Gemcitabine-induced thrombotic microangiopathy: a systematic review Nephrol. Dial. Transplant., November 1, 2006; 21(11): 3038 - 3045. [Full Text] [PDF]

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