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Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty
A-71-year-old woman was diagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) accompanied by essential thrombocythemia (ET) with JAK2 V617F mutation. Blood test showed remarkable increase of platelet counts (132.9 × 10^4/μL) and elevated plasma BNP level (125.1pg/mL). Right heart cathet...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7528185/ https://www.ncbi.nlm.nih.gov/pubmed/33024690 http://dx.doi.org/10.1016/j.rmcr.2020.101235 |
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author | Yamaguchi, Tomohiro Izumiya, Yasuhiro Hayashi, Hiroya Ichikawa, Yoshitaka Ishikawa, Hirotoshi Shibata, Atsushi Yamazaki, Takanori Yoshiyama, Minoru |
author_facet | Yamaguchi, Tomohiro Izumiya, Yasuhiro Hayashi, Hiroya Ichikawa, Yoshitaka Ishikawa, Hirotoshi Shibata, Atsushi Yamazaki, Takanori Yoshiyama, Minoru |
author_sort | Yamaguchi, Tomohiro |
collection | PubMed |
description | A-71-year-old woman was diagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) accompanied by essential thrombocythemia (ET) with JAK2 V617F mutation. Blood test showed remarkable increase of platelet counts (132.9 × 10^4/μL) and elevated plasma BNP level (125.1pg/mL). Right heart catheterization (RHC) revealed remarkably high mean pulmonary arterial pressure (mPAP) of 43 mmHg. We gave her riociguat of 7.5mg, oral anticoagulants, oxygen inhalation for CTEPH, and anagrelide for ET. We performed 4 sessions of balloon pulmonary angioplasty (BPA) in 9 months RHC revealed successful hemodynamic improvement (mPAP = 21 mmHg) after final BPA procedure without riociguat. At six month later after final BPA procedure, RHC showed steadily improvement of mPAP (21 mmHg) without riociguat and oxygen inhalation. She lives well without oxygen inhalation and PH targeted therapy. This is the first report of successful treatment for a patient with CTEPH comorbid with ET with JAK2 V617F mutation by BPA. |
format | Online Article Text |
id | pubmed-7528185 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-75281852020-10-05 Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty Yamaguchi, Tomohiro Izumiya, Yasuhiro Hayashi, Hiroya Ichikawa, Yoshitaka Ishikawa, Hirotoshi Shibata, Atsushi Yamazaki, Takanori Yoshiyama, Minoru Respir Med Case Rep Case Report A-71-year-old woman was diagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) accompanied by essential thrombocythemia (ET) with JAK2 V617F mutation. Blood test showed remarkable increase of platelet counts (132.9 × 10^4/μL) and elevated plasma BNP level (125.1pg/mL). Right heart catheterization (RHC) revealed remarkably high mean pulmonary arterial pressure (mPAP) of 43 mmHg. We gave her riociguat of 7.5mg, oral anticoagulants, oxygen inhalation for CTEPH, and anagrelide for ET. We performed 4 sessions of balloon pulmonary angioplasty (BPA) in 9 months RHC revealed successful hemodynamic improvement (mPAP = 21 mmHg) after final BPA procedure without riociguat. At six month later after final BPA procedure, RHC showed steadily improvement of mPAP (21 mmHg) without riociguat and oxygen inhalation. She lives well without oxygen inhalation and PH targeted therapy. This is the first report of successful treatment for a patient with CTEPH comorbid with ET with JAK2 V617F mutation by BPA. Elsevier 2020-09-28 /pmc/articles/PMC7528185/ /pubmed/33024690 http://dx.doi.org/10.1016/j.rmcr.2020.101235 Text en © 2020 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Yamaguchi, Tomohiro Izumiya, Yasuhiro Hayashi, Hiroya Ichikawa, Yoshitaka Ishikawa, Hirotoshi Shibata, Atsushi Yamazaki, Takanori Yoshiyama, Minoru Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title | Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title_full | Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title_fullStr | Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title_full_unstemmed | Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title_short | Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty |
title_sort | successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the jak2 v617f mutation by balloon pulmonary angioplasty |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7528185/ https://www.ncbi.nlm.nih.gov/pubmed/33024690 http://dx.doi.org/10.1016/j.rmcr.2020.101235 |
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