The cancer paradigm of severe pulmonary arterial hypertension
The cancer paradigm of severe pulmonary arterial hypertension
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DOI：
10.1164/rccm.200709-1369pp
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发表时间：
2008-09-15
影响因子：
24.7
通讯作者：
Voelkel, Norbert F.
中科院分区：
文献类型：
--
作者：
Rai, Pradeep R.;Cool, Carlyne D.;Voelkel, Norbert F.
无论是特发性还是继发性，重度肺动脉高压（PAH）的丛状病变在组织学表现上相似。这两种形式的疾病都表现出活跃的内皮细胞增殖，而没有凋亡的证据。在这里，我们讨论的病理生物学的非典型性，血管增生性内皮细胞在严重的PAH。内皮细胞作为“准恶性”细胞的概念为重度PAH的抗增殖、抗血管生成治疗提供了新的框架。
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.