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Prepublished online as a Blood First Edition Paper on April 17, 2002; DOI 10.1182/blood-2002-02-0344. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-02-0344v1 100/2/710    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 Bianchi, V. Articles by Lämmle, B. Search for Related Content PubMed PubMed Citation Articles by Bianchi, V. Articles by Lämmle, B. Related Collections Hemostasis, Thrombosis, and Vascular Biology Brief Reports Clinical Trials and Observations Related Letter in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 July 2002, Vol. 100, No. 2, pp. 710-713 BRIEF REPORT Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura Valentina Bianchi, Rodolfo Robles, Lorenzo Alberio, Miha Furlan, and Bernhard Lämmle From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland. A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic and other conditions. We studied 68 patients with thrombocytopenia due to severe sepsis or septic shock (n = 17), heparin-induced thrombocytopenia (n = 16), idiopathic thrombocytopenic purpura (n = 10), or other hematologic (n = 15) or miscellaneous conditions (n = 10). Twelve of the 68 patients had subnormal levels of ADAMTS13 activity ( 30%), but none had less than 10%. Thus, the study showed that ADAMTS13 activity is decreased in a substantial proportion of patients with thrombocytopenia of various causes. A severe deficiency of ADAMTS13 (< 5%), identified in more than 120 patients during 1996 to 2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled TTP. © 2002 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Letter in Blood Online: Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura Han-Mou Tsai, Bernhard Lämmle, Valentina Bianchi, Lorenzo Alberio, Miha Furlan, Giuseppe Remuzzi, Miriam Galbusera, and Pier Mannuccio Mannucci Blood 2002 100: 3839-3842. [Full Text] [PDF] 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] C. L. Bockmeyer, R. A. Claus, U. Budde, K. Kentouche, R. Schneppenheim, W. Losche, K. Reinhart, and F. M. Brunkhorst Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor Haematologica, January 1, 2008; 93(1): 137 - 140. [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. 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Sakata Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure Blood, January 15, 2006; 107(2): 528 - 534. [Abstract] [Full Text] [PDF] W. Zhou, L. Dong, D. Ginsburg, E. E. Bouhassira, and H.-M. Tsai Enzymatically Active ADAMTS13 Variants Are Not Inhibited by Anti-ADAMTS13 Autoantibodies: A NOVEL THERAPEUTIC STRATEGY? J. Biol. Chem., December 2, 2005; 280(48): 39934 - 39941. [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] G. G. Levy, D. G. Motto, and D. Ginsburg ADAMTS13 turns 3 Blood, July 1, 2005; 106(1): 11 - 17. [Abstract] [Full Text] [PDF] G. Wolf Not known from ADAM(TS-13)--novel insights into the pathophysiology of thrombotic microangiopathies Nephrol. Dial. Transplant., July 1, 2004; 19(7): 1687 - 1693. [Full Text] [PDF] K. Kokame, M. Matsumoto, Y. Fujimura, and T. Miyata VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13 Blood, January 15, 2004; 103(2): 607 - 612. [Abstract] [Full Text] [PDF] J. E. Sadler, J. L. Moake, T. Miyata, and J. N. George Recent Advances in Thrombotic Thrombocytopenic Purpura Hematology, January 1, 2004; 2004(1): 407 - 423. [Abstract] [Full Text] [PDF] F. Vincent, M.-A. Costa, E. Rondeau, S. K. Vesely, and J. N. George ADAMTS13 and thrombotic thrombocytopenic purpura-hemolytic uremic syndrome Blood, November 15, 2003; 102(10): 3848 - 3849. [Full Text] [PDF] F. Scheiflinger, P. Knobl, B. Trattner, B. Plaimauer, G. Mohr, M. Dockal, F. Dorner, and M. 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[Abstract] [Full Text] [PDF] H.-M. Tsai Platelet Activation and the Formation of the Platelet Plug: Deficiency of ADAMTS13 Causes Thrombotic Thrombocytopenic Purpura Arterioscler. Thromb. Vasc. Biol., March 1, 2003; 23(3): 388 - 396. [Abstract] [Full Text] [PDF] R. Schneppenheim, U. Budde, F. Oyen, D. Angerhaus, V. Aumann, E. Drewke, W. Hassenpflug, J. Haberle, K. Kentouche, E. Kohne, et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP Blood, March 1, 2003; 101(5): 1845 - 1850. [Abstract] [Full Text] [PDF] J. N. George and S. K. Vesely Thrombotic Thrombocytopenic Purpura: From the Bench to the Bedside, but Not Yet to the Community Ann Intern Med, January 21, 2003; 138(2): 152 - 153. [Full Text] [PDF] H.-M. Tsai, B. Lammle, V. Bianchi, L. Alberio, M. Furlan, G. Remuzzi, M. Galbusera, and P. M. Mannucci Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura Blood, November 15, 2002; 100(10): 3839 - 3842. [Full Text] [PDF] J. N. George, J. E. 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