ID | 114689 |
Title Alternative | A Case of Chronic Thromboembolic Pulmonary Hypertension Secondary to Myeloproliferative Disease
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Author |
Shimura, Takuya
Tokushima University
Saijo, Yoshihito
Tokushima University
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Yagi, Shusuke
Tokushima University
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Kusunose, Kenya
Tokushima University
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Yamada, Hirotsugu
Tokushima University
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Fujimoto, Yuta
Tokushima University
Matsumoto, Kazuhisa
Tokushima University
Kawabata, Yutaka
Tokushima University
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Yamada, Nao
Tokushima University
Ise, Takayuki
Tokushima University
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Yamaguchi, Koji
Tokushima University
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Soeki, Takeshi
Tokushima University
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Wakatsuki, Tetsuzo
Tokushima University
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Sata, Masataka
Tokushima University
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Keywords | Chronic Thromboembolic Pulmonary Hypertension
pulmonary hypertension
Myelofibrosis
Pulmonary embolism
Deep venous thrombosis
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Content Type |
Journal Article
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Description | A woman in her 60s presented with shortness of breath on exertion and was admitted to a nearby hospital in March 200X. Contrast-enhanced computer tomography scan showed contrast defect images in the pulmonary artery and lower extremity vein. She was diagnosed with pulmonary embolism and deep venous thrombosis and anticoagulant therapy was started. At the same time, a blood test revealed an abnormal increased platelet count(740,000/μl), and she was diagnosed as myeloproliferative disease(primary myelofibrosis, JAK2 mutation +). We follow up with oral administration of a steroid because she had a low risk of primary myelofibrosis. However, the symptom had been lasting, she was admitted into our hospital for examining the origin of symptom and treatment. Cardiac echocardiography suggested the presence of pulmonary hypertension, and lung ventilation perfusion scintigraphy showed widespread wedge accumulation defect, depressed area in bilateral lungs, and ventilator blood flow mismatch. In cardiac catheterization, the mean pulmonary artery pressure was as high as 37mmHg. Per the test results, she was diagnosed chronic thromboembolic pulmonary hypertension(CTEPH)secondary to primary myelofibrosis. We proposed invasive treatment(pulmonary artery endarterectomy, balloon pulmonary arterioplasty), but she desired just oxygen administration and medication therapy.
It is reported that CTEPH develops in an organized thrombus after acute pulmonary embolism, but the mechanism of that development has not been revealed. In this case with primary myelofibrosis, we consider that the decrease of pulmonary vascular bed is due to a blood cell disorder and vascular remodeling is due to an increase of vascular endothelial growth factor and platelet derived growth factor secreted by abnormal increased platelet contributed to elevation of pulmonary artery pressure. |
Journal Title |
Shikoku Acta Medica
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ISSN | 00373699
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NCID | AN00102041
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Publisher | 徳島医学会
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Volume | 75
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Issue | 5-6
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Start Page | 227
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End Page | 232
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Sort Key | 227
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Published Date | 2019-12-25
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FullText File | |
language |
jpn
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TextVersion |
Publisher
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departments |
Medical Sciences
University Hospital
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