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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4043-4049. Prepublished online as a Blood First Edition Paper on February 24, 2004; DOI 10.1182/blood-2003-11-4035. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-11-4035v1 103/11/4043    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 Zheng, X. L. Articles by Sadler, J. E. Search for Related Content PubMed PubMed Citation Articles by Zheng, X. L. Articles by Sadler, J. E. Related Collections Hemostasis, Thrombosis, and Vascular Biology Free Research Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura X. Long Zheng, Richard M. Kaufman, Lawrence T. Goodnough, and J. Evan Sadler From the Department of Pathology and Immunology and Department of Medicine, Barnes-Jewish Hospital, St Louis, MO; Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO; and Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, PA. Therapeutic plasma exchange is an effective empiric treatment for thrombotic thrombocytopenic purpura (TTP), but how therapy affects the level of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) or inhibitor has not been reported in many patients. We prospectively analyzed ADAMTS13 activity and inhibitor levels in 37 adults with TTP. ADAMTS13 level at presentation was lower than 5% in 16 of 20 patients with idiopathic TTP and in none of 17 patients with TTP associated with hematopoietic stem cell transplantation, cancer, drugs, or pregnancy (P < .00001). Seven of the 16 patients with ADAMTS13 activity lower than 5% ( 44%) had inhibitors. For 8 patients followed serially with ADAMTS13 activity lower than 5% but no inhibitor at presentation, plasma exchange led to complete clinical remission and a rise in ADAMTS13 level. In contrast, 4 patients with low ADAMTS13 activity but high-titer inhibitor (> 5 units/mL) had neither a rise in ADAMTS13 activity nor a reduction in the inhibitor titer: 3 had recurrent disease and 1 died. Among 17 patients with AD-AMTS13 activity at presentation higher than 25%, 10 died. Mortality rate for idiopathic TTP was 15%, whereas mortality for nonidiopathic TTP was 59% (P < .02). We conclude that assays of ADAMTS13 activity and inhibitors in addition to the clinical categories (idiopathic TTP and nonidiopathic TTP) are predictive of outcome and may be useful to tailor patient treatment. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: ADAMTS13 and TTP: the clot thickens James N. George and Sara K. Vesely Blood 2004 103: 3997-3998. [Full Text] [PDF] This article has been cited by other articles: J. E. 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[Abstract] [Full Text] [PDF] C. L. Bennett, B. Kim, A. Zakarija, N. Bandarenko, D. K. Pandey, C. G. Buffie, J. M. McKoy, A. D. Tevar, J. F. Cursio, P. R. Yarnold, et al. Two Mechanistic Pathways for Thienopyridine-Associated Thrombotic Thrombocytopenic Purpura: A Report From the SERF-TTP Research Group and the RADAR Project J. Am. Coll. Cardiol., September 18, 2007; 50(12): 1138 - 1143. [Abstract] [Full Text] [PDF] P. Zhang, W. Pan, A. H. Rux, B. S. Sachais, and X. L. Zheng The cooperative activity between the carboxyl-terminal TSP1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow Blood, September 15, 2007; 110(6): 1887 - 1894. [Abstract] [Full Text] [PDF] P. M. Mannucci Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome: much progress and many remaining issues Haematologica, July 1, 2007; 92(7): 878 - 880. [Full Text] [PDF] S. Ferrari, F. Scheiflinger, M. Rieger, G. Mudde, M. Wolf, P. Coppo, J.-P. Girma, E. 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