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Blood, 25 June 2009, Vol. 113, No. 26, pp. 6511-6521. Prepublished online as a Blood First Edition Paper on April 24, 2009; DOI 10.1182/blood-2009-01-129155. This Article Full Text (PDF) Supplemental Reference All Versions of this Article: blood-2009-01-129155v1 113/26/6511    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 Cines, D. B. Articles by Luning Prak, E. T. Search for Related Content PubMed PubMed Citation Articles by Cines, D. B. Articles by Luning Prak, E. T. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted January 7, 2009 Accepted March 19, 2009 The ITP syndrome: pathogenic and clinical diversity Douglas B. Cines*, James B. Bussel, Howard A. Liebman, and Eline T. Luning Prak Department of Pathology of Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, United States Departments of Pediatrics and Obstetrics-Gynecology, and Medicine, Weill Medical Center, College of Cornell University, New York, NY, United States Department of Medicine, University of Southern California, Los Angeles, CA, United States * Corresponding author; email: dcines{at}mail.med.upenn.edu. Immune thrombocytopenia (ITP) is mediated by platelet autoantibodies that accelerate platelet destruction and inhibit their production. Most cases are considered idiopathic, whereas others are secondary to co-existing conditions. Insights from secondary forms suggest the proclivity to develop platelet-reactive antibodies arises through diverse mechanisms. Variability in natural history and response to therapy suggests that primary ITP is also heterogeneous. Certain cases may be secondary to persistent, sometimes inapparent, infections, accompanied by co-existing antibodies that influence outcome. Alternatively, underlying immunodeficiences may emerge. Additionally, environmental and genetic factors may impact platelet turnover, propensity to bleed and response to ITP-directed therapy. We review the pathophysiology of several common secondary forms of ITP. We suggest that primary ITP is best thought of as an autoimmune syndrome. Better understanding of pathogenesis and tolerance checkpoint defects leading to autoantibody formation may facilitate patient-specific approaches to diagnosis and management. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Rodeghiero, M. Ruggeri, and on behalf of the International Working Group Response:Immune thrombocytopenic purpura: terminology and definitions Blood, August 27, 2009; 114(9): 2004 - 2004. [Full Text] [PDF]

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