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Blood, 1 July 2008, Vol. 112, No. 1, pp. 11-18. This Article Full Text Full Text (PDF) 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 CrossRef Google Scholar Articles by Sadler, J. E. PubMed PubMed Citation Articles by Sadler, J. E. Related Collections Hemostasis, Thrombosis, and Vascular Biology Free Research Articles ASH 50th Anniversary Reviews Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  ASH 50TH ANNIVERSARY REVIEW Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura J. Evan Sadler1 1 Department of Medicine, Department of Biochemistry and Molecular Biophysics, and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO Discoveries during the past decade have revolutionized our understanding of idiopathic thrombotic thrombocytopenic purpura (TTP). Most cases in adults are caused by acquired autoantibodies that inhibit ADAMTS13, a metalloprotease that cleaves von Willebrand factor within nascent platelet-rich thrombi to prevent hemolysis, thrombocytopenia, and tissue infarction. Although approximately 80% of patients respond to plasma exchange, which removes autoantibody and replenishes ADAMTS13, one third to one half of survivors develop refractory or relapsing disease. Intensive immunosuppressive therapy with rituximab appears to be effective as salvage therapy, and ongoing clinical trials should determine whether adjuvant rituximab with plasma exchange also is beneficial at first diagnosis. A major unanswered question is whether plasma exchange is effective for the subset of patients with idiopathic TTP who do not have severe ADAMTS13 deficiency. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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