Platelet activation in cystic fibrosis

O'Sullivan, B, Linden, M, Frelinger, A, Barnard, M, Spencer-Manzon, M, Morris, J, Salem, R, Laposata, M and Michelson, A 2005, 'Platelet activation in cystic fibrosis', Blood, vol. 105, no. 12, pp. 4635-4641.


Document type: Journal Article
Collection: Journal Articles

Title Platelet activation in cystic fibrosis
Author(s) O'Sullivan, B
Linden, M
Frelinger, A
Barnard, M
Spencer-Manzon, M
Morris, J
Salem, R
Laposata, M
Michelson, A
Year 2005
Journal name Blood
Volume number 105
Issue number 12
Start page 4635
End page 4641
Total pages 7
Publisher American Society of Hematology
Abstract 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.
Subject Clinical Sciences not elsewhere classified
DOI - identifier 10.1182/blood-2004-06-2098
ISSN 0006-4971
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