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Blood, 15 June 2005, Vol. 105, No. 12, pp. 4635-4641. Prepublished online as a Blood First Edition Paper on February 10, 2005; DOI 10.1182/blood-2004-06-2098. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-06-2098v1 105/12/4635    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 O'Sullivan, B. P. Articles by Michelson, A. D. Search for Related Content PubMed PubMed Citation Articles by O'Sullivan, B. P. Articles by Michelson, A. D. Related Collections Hemostasis, Thrombosis, and Vascular Biology Signal Transduction Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Platelet activation in cystic fibrosis Brian P. O'Sullivan, Matthew D. Linden, Andrew L. Frelinger, III, Marc R. Barnard, Michele Spencer-Manzon, James E. Morris, Raneem O. Salem, Michael Laposata, and Alan D. Michelson From the Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA; the Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA; the Department of Molecular Biology, Genzyme, Framingham, MA; and the Division of Laboratory Medicine, Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA. Cystic fibrosis (CF) is caused by a mutation of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). We examined platelet function in CF patients because lung inflammation is part of this disease and platelets contribute to inflammation. CF patients had increased circulating leukocyte-platelet aggregates and increased platelet responsiveness to agonists compared with healthy controls. CF plasma caused activation of normal and CF platelets; however, activation was greater in CF platelets. Furthermore, washed CF platelets also showed increased reactivity to agonists. CF platelet hyperreactivity was incompletely inhibited by prostaglandin E1 (PGE1). As demonstrated by Western blotting and reverse-transcriptase-polymerase chain reaction (RT-PCR), there was neither CFTR nor CFTR-specific mRNA in normal platelets. There were abnormalities in the fatty acid composition of membrane fractions of CF platelets. In summary, CF patients have an increase in circulating activated platelets and platelet reactivity, as determined by monocyte-platelet aggregation, neutrophil-platelet aggregation, and platelet surface P-selectin. This increased platelet activation in CF is the result of both a plasma factor(s) and an intrinsic platelet mechanism via cyclic adenosine monophosphate (cAMP)/adenylate cyclase, but not via platelet CFTR. Our findings may account, at least in part, for the beneficial effects of ibuprofen in CF. (Blood. 2005;105:4635-4641) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: B. P. O'Sullivan and A. D. Michelson The Inflammatory Role of Platelets in Cystic Fibrosis Am. J. Respir. Crit. Care Med., March 1, 2006; 173(5): 483 - 490. [Abstract] [Full Text] [PDF] V. S. Dole, W. Bergmeier, H. A. Mitchell, S. C. Eichenberger, and D. D. Wagner Activated platelets induce Weibel-Palade-body secretion and leukocyte rolling in vivo: role of P-selectin Blood, October 1, 2005; 106(7): 2334 - 2339. [Abstract] [Full Text] [PDF]

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