The cancer paradigm of severe pulmonary arterial hypertension.
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.
Department of Pathology, University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA.
Pradeep R
PR
Rai
Carlyne D
CD
Cool
Judy A C
JA
King
Troy
T
Stevens
Nana
N
Burns
Robert A
RA
Winn
Michael
M
Kasper
Norbert F
NF
Voelkel
D049109:Cell Proliferation; D042783:Endothelial Cells / Q000473:pathology / Q000502:physiology; D005786:Gene Expression Regulation / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D007150:Immunohistochemistry; D009369:Neoplasms / Q000503:physiopathology; D011651:Pulmonary Artery / Q000473:pathology; D009626:Terminology as Topic; D014661:Vasoconstriction / Q000502:physiology; D051153:Wnt Proteins / Q000378:metabolism
C092778:WNT7A protein, human; D051153:Wnt Proteins