Journal
CARDIOVASCULAR PATHOLOGY
Volume 15, Issue 2, Pages 91-99Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.carpath.2005.11.006
Keywords
pulmonary vein stenosis; myofibroblasts; receptor tyrosine kinases
Categories
Funding
- NHLBI NIH HHS [R01 HL035716, P50 HL74734] Funding Source: Medline
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Background: 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. Methods: Seven PVS cases were studied with antibodies for smooth muscle actin (SMA), muscle-specific actin (MSA), monoclonal desmin, S 100 protein, CD31, CD34, CD45RO, CD68, CD99, Ki-67 (MIB-1), and with antibodies directed against several receptor tyrosine kinases (RTK), including platelet-derived growth factor alpha and beta receptor (PDGFR-alpha and -beta), epidermal growth factor receptor (EGFR), fibroblast growth factor receptor (FGFR), vascular endothelial growth factor I and 2 receptor (VEGFR), and stem cell factor receptor (c-kit). Results: 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-alpha and -beta, FGFR, and VEGFR-2. Lesional cells stained for VEGF and PDGF beta receptor was phosphorylated. Conclusions: 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. (C) 2006 Elsevier Inc. All rights reserved.
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