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ID 114689
タイトル別表記
A Case of Chronic Thromboembolic Pulmonary Hypertension Secondary to Myeloproliferative Disease
著者
志村, 拓哉 徳島大学
西條, 良仁 徳島大学
藤本, 裕太 徳島大学
松本, 和久 徳島大学
上野, 理絵 徳島大学
川端, 豊 徳島大学
山田, なお 徳島大学
キーワード
Chronic Thromboembolic Pulmonary Hypertension
pulmonary hypertension
Myelofibrosis
Pulmonary embolism
Deep venous thrombosis
資料タイプ
学術雑誌論文
抄録
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.
掲載誌名
四国医学雑誌
ISSN
00373699
cat書誌ID
AN00102041
出版者
徳島医学会
75
5-6
開始ページ
227
終了ページ
232
並び順
227
発行日
2019-12-25
フルテキストファイル
言語
jpn
著者版フラグ
出版社版
部局
医学系
病院