Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells

Primary pulmonary vein stenosis (PVS) is a progressive disorder of infants. Although catheter based intervention and chemotherapy are used to manage the disorder, the benefit of these approaches is reduced considerably by restenosis. The nature of the intimal cells causing the occlusive lesions in P...

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Bibliographic Details
Published in:Cardiovascular pathology 2006-03, Vol.15 (2), p.91-99
Main Authors: Riedlinger, Wolfram F.J., Juraszek, Amy L., Jenkins, Kathy J., Nugent, Alan W., Balasubramanian, Sowmya, Calicchio, Monica L., Kieran, Mark W., Collins, Tucker
Format: Article
Language:English
Subjects:
Animals
Constriction, Pathologic - enzymology
Female
Humans
Immunohistochemistry
Infant
Infant, Newborn
Male
Myofibroblasts
Pulmonary vein stenosis
Pulmonary Veins - pathology
Rabbits
Receptor Protein-Tyrosine Kinases - metabolism
Receptor tyrosine kinases
Tunica Intima - pathology
Vascular Diseases - enzymology
Vascular Diseases - pathology
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Summary:Primary pulmonary vein stenosis (PVS) is a progressive disorder of infants. Although catheter based intervention and chemotherapy are used to manage the disorder, the benefit of these approaches is reduced considerably by restenosis. The nature of the intimal cells causing the occlusive lesions in PVS is poorly understood. Seven PVS cases were studied with antibodies for smooth muscle actin (SMA), muscle-specific actin (MSA), monoclonal desmin, S100 protein, CD31, CD34, CD45RO, CD68, CD99, Ki-67 (MIB-I), and with antibodies directed against several receptor tyrosine kinases (RTK), including platelet-derived growth factor alpha and beta receptor (PDGFR-α and -β), epidermal growth factor receptor (EGFR), fibroblast growth factor receptor (FGFR), vascular endothelial growth factor 1 and 2 receptor (VEGFR), and stem cell factor receptor (c-kit). Lesional cells stained strongly and diffusely with SMA and MSA, but not for macrophage, lymphocyte, endothelial markers, or for Ki-67. RTK expression was strong and diffuse for PDGFR-α and -β, FGFR, and VEGFR-2. Lesional cells stained for VEGF and PDGF β receptor was phosphorylated. The histologic appearance, and the strong diffuse immunoreactivity for smooth muscle markers, indicates that the intimal lesional cells are myofibroblast-like. Expression of various receptor tyrosine kinases and some ligands suggests an autocrine or paracrine role of these proteins in the pathogenesis of the intimal occlusive lesion in PVS.
ISSN:1054-8807
1879-1336
DOI:10.1016/j.carpath.2005.11.006