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Am J Respir Crit Care Med. 2008 Sep 15;178(6):558-64. doi: 10.1164/rccm.200709-1369PP. Epub 2008 Jun 12.

The cancer paradigm of severe pulmonary arterial hypertension.

Author information

1
Department of Pathology, University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA.

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.

PMID:
18556624
PMCID:
PMC2542431
DOI:
10.1164/rccm.200709-1369PP
[Indexed for MEDLINE]
Free PMC Article

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