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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...

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Autores principales: Ohashi, Kana, Nishimura, Rintaro, Sugimoto, Shunsuke, Sakao, Seiichiro, Tanabe, Nobuhiro, Tatsumi, Koichiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2019
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.
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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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