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Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley & Sons, Ltd
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391640/ https://www.ncbi.nlm.nih.gov/pubmed/30847222 http://dx.doi.org/10.1002/rcr2.411 |
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author | Ohashi, Kana Nishimura, Rintaro Sugimoto, Shunsuke Sakao, Seiichiro Tanabe, Nobuhiro Tatsumi, Koichiro |
author_facet | Ohashi, Kana Nishimura, Rintaro Sugimoto, Shunsuke Sakao, Seiichiro Tanabe, Nobuhiro Tatsumi, Koichiro |
author_sort | Ohashi, Kana |
collection | PubMed |
description | Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated with POEMS syndrome. She was started on dexamethasone and thalidomide. Although, the plasma vascular endothelial growth factor (VEGF) level decreased, systolic pulmonary artery pressure (sPAP) remained high. Auto‐peripheral blood stem cell transplantation improved the plasma VEGF and sPAP levels. Four years later, she presented with dyspnoea on exertion, and elevated plasma VEGF and sPAP levels. Subsequently, on administering sildenafil and macitentan, the plasma VEGF and PH levels improved. Pulmonary vasodilators can be considered when PH remains after treatment of POEMS syndrome. |
format | Online Article Text |
id | pubmed-6391640 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | John Wiley & Sons, Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-63916402019-03-07 Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome Ohashi, Kana Nishimura, Rintaro Sugimoto, Shunsuke Sakao, Seiichiro Tanabe, Nobuhiro Tatsumi, Koichiro Respirol Case Rep Case Reports Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated with POEMS syndrome. She was started on dexamethasone and thalidomide. Although, the plasma vascular endothelial growth factor (VEGF) level decreased, systolic pulmonary artery pressure (sPAP) remained high. Auto‐peripheral blood stem cell transplantation improved the plasma VEGF and sPAP levels. Four years later, she presented with dyspnoea on exertion, and elevated plasma VEGF and sPAP levels. Subsequently, on administering sildenafil and macitentan, the plasma VEGF and PH levels improved. Pulmonary vasodilators can be considered when PH remains after treatment of POEMS syndrome. John Wiley & Sons, Ltd 2019-02-27 /pmc/articles/PMC6391640/ /pubmed/30847222 http://dx.doi.org/10.1002/rcr2.411 Text en © 2019 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Case Reports Ohashi, Kana Nishimura, Rintaro Sugimoto, Shunsuke Sakao, Seiichiro Tanabe, Nobuhiro Tatsumi, Koichiro Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title | Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title_full | Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title_fullStr | Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title_full_unstemmed | Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title_short | Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome |
title_sort | effectiveness of pulmonary vasodilators on pulmonary hypertension associated with poems syndrome |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391640/ https://www.ncbi.nlm.nih.gov/pubmed/30847222 http://dx.doi.org/10.1002/rcr2.411 |
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