Published ahead of print on June 12, 2008, doi:10.1164/rccm.200709-1369PP
© 2008 American Thoracic Society doi: 10.1164/rccm.200709-1369PP
The Cancer Paradigm of Severe Pulmonary Arterial Hypertension1 Department of Pathology, University of Colorado at Denver and Health Sciences Center, Denver, Colorado; 2 Center for Lung Biology, University of South Alabama, Mobile, Alabama; 3 Pulmonary and Critical Care Medicine, University of Colorado at Denver and Health Sciences Center, Denver, Colorado; 4 Technische Hochschule, Gustav Carus Universität, Dresden, Germany; and 5 Pulmonary and Critical Care Medicine Division, Virginia Commonwealth University, Richmond, Virginia Correspondence and requests for reprints should be addressed to Norbert F. Voelkel, M.D., Professor of Medicine, Pulmonary and Critical Care Medicine Division, Virginia Commonwealth University, Sanger Hall, 7-020, 1101 E. Marshall St., Richmond, VA 23298. E-mail: nvoelkel{at}mcvh-vcu.edu ABSTRACT The plexiform lesions of severe pulmonary arterial hypertension (PAH) are similar in histologic appearance, whether the disease is idiopathic or secondary. Both forms of the disease show actively proliferating endothelial cells without evidence of apoptosis. Here, we discuss the pathobiology of the atypical, angioproliferative endothelial cells in severe PAH. The concept of the endothelial cell as a "quasi-malignant" cell provides a new framework for antiproliferative, antiangiogenic therapy in severe PAH.
Key Words: pulmonary arterial hypertension • plexiform lesions • angiogenesis • cancer Related articles in AJRCCM:
This article has been cited by other articles:
|
|
|||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
