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Research Article Free access | 10.1172/JCI1910

Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension.

S D Lee, K R Shroyer, N E Markham, C D Cool, N F Voelkel, and R M Tuder

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Lee, S. in: PubMed | Google Scholar

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Shroyer, K. in: PubMed | Google Scholar

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Markham, N. in: PubMed | Google Scholar

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Cool, C. in: PubMed | Google Scholar

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Voelkel, N. in: PubMed | Google Scholar

Department of Pathology,, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA.

Find articles by Tuder, R. in: PubMed | Google Scholar

Published March 1, 1998 - More info

Published in Volume 101, Issue 5 on March 1, 1998
J Clin Invest. 1998;101(5):927–934. https://doi.org/10.1172/JCI1910.
© 1998 The American Society for Clinical Investigation
Published March 1, 1998 - Version history
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Abstract

The etiology and pathogenesis of the vascular lesions characterizing primary pulmonary hypertension (PPH), an often fatal pulmonary vascular disease, are largely unknown. Plexiform lesions composed of proliferating endothelial cells occur in between 20 and 80% of the cases of this irreversible pulmonary vascular disease. Recently, technology to assess monoclonality has allowed the distinction between cellular proliferation present in neoplasms from that in reactive nonneoplastic tissue. To determine whether the endothelial cell proliferation in plexiform lesions in PPH is monoclonal or polyclonal, we assessed the methylation pattern of the human androgen receptor gene by PCR (HUMARA) in proliferated endothelial cells in plexiform lesions from female PPH patients (n = 4) compared with secondary pulmonary hypertension (PH) patients (n = 4). In PPH, 17 of 22 lesions (77%) were monoclonal. However, in secondary PH, all 19 lesions examined were polyclonal. Smooth muscle cell hyperplasia in pulmonary vessels (n = 11) in PPH and secondary PH was polyclonal in all but one of the examined vessels. The monoclonal expansion of endothelial cells provides the first marker that allows the distinction between primary and secondary PH. Our data of a frequent monoclonal endothelial cell proliferation in PPH suggests that a somatic genetic alteration similar to that present in neoplastic processes may be responsible for the pathogenesis of PPH.

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