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Published ahead of print on June 12, 2008, doi:10.1164/rccm.200709-1369PP

Am. J. Respir. Crit. Care Med., Volume 178, Number 6, September 2008, 558-564

A more recent version of this article appeared on September 15, 2008
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Submitted on September 14, 2007
Accepted on June 12, 2008

The Cancer Paradigm of Severe Angioproliferative Pulmonary Hypertension

Pradeep R Rai1, Carlyne D Cool1, Judy AC King2, Troy Stevens2, Nana Burns3, Robert A Winn3, Michael Kasper4, and Norbert F Voelkel3*

1 Department of Pulmonary and Critical Care Medicine, and Department of Pathology, University of Colorado at Denver and Health Sciences Center, Denver, CO, USA, 2 University of South Alabama, Center for Lung Biology, Montgomery, AL, USA, 3 Department of Pulmonary and Critical Care Medicine, UCDHSC, Denver, CO, USA, 4 Department of Anatomy, Technical University of Dresden, Dresden, Germany

* To whom correspondence should be addressed. E-mail: nvoelkel{at}mcvh-vcu.edu.

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: severe pulmonary hypertension, angioproliferation, plexiform lesions




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