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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 (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 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 Lammle, B. Search for Related Content PubMed PubMed Citation Articles by Bianchi, V. Articles by Lammle, B. Related Collections Related Letter in Blood Online Social Bookmarking                   What's this? Submitted February 4, 2002 Accepted March 6, 2002 Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura (TTP) Valentina Bianchi, Rodolfo Robles, Lorenzo Alberio, Miha Furlan, and Bernhard Lammle* * Corresponding author; email: bernhard.laemmle{at}insel.ch. A severely deficient von Willebrand factor-cleaving protease (ADAMTS13) activity (<5% of normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP), but not in those having been diagnosed with hemolytic uremic syndrome. However, ADAMTS13 deficiency has recently been claimed not to be specific for TTP, being found in various thrombocytopenic and other conditions. We performed a study on 68 patients with thrombocytopenia due to severe sepsis/septic shock (n=17), heparin-induced thombocytopenia (n=16), idiopathic thrombocytopenic purpura (n=10), other hematologic (n=15) and miscellaneous conditions (n=10). Twelve of the 68 patients showed subnormal ADAMTS13 activity (A severely deficient von Willebrand factor-cleaving protease (ADAMTS13) activity (<5% of normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP), but not in those having been diagnosed with hemolytic uremic syndrome. However, ADAMTS13 deficiency has recently been claimed not to be specific for TTP, being found in various thrombocytopenic and other conditions. We performed a study on 68 patients with thrombocytopenia due to severe sepsis/septic shock (n=17), heparin-induced thombocytopenia (n=16), idiopathic thrombocytopenic purpura (n=10), other hematologic (n=15) and miscellaneous conditions (n=10). Twelve of the 68 patients showed subnormal ADAMTS13 activity (30%), but none <10%. This study shows that ADAMTS13 activity is decreased in a substantial proportion of patients with thrombocytopenia of various etiologies. A severe deficiency of ADAMTS13 (<5%), identified in more than 120 patients during 1996-2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled as TTP. 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: A. H.M. Reid, A. Protheroe, G. Attard, N. Hayward, L. Vidal, J. Spicer, H. M. Shaw, E. A. Bone, J. Carter, L. Hooftman, et al. 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