Imatinib is Partially Effective for the Treatment of Pulmonary Capillary Hemangiomatosis
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- Adachi Shiro
- Department of Cardiology, Nagoya University Graduate School of Medicine, Japan
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- Hirashiki Akihiro
- Department of Advanced Medicine in Cardiopulmonary Disease, Nagoya University Graduate School of Medicine, Japan
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- Kondo Takahisa
- Department of Advanced Medicine in Cardiopulmonary Disease, Nagoya University Graduate School of Medicine, Japan
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- Nakaguro Masato
- Department of Pathology, Nagoya University Graduate School of Medicine, Japan
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- Ogawa Aiko
- Department of Cardiology, National Hospital Organization Okayama Medical Center, Japan
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- Miyaji Katsumasa
- Department of Cardiology, National Hospital Organization Okayama Medical Center, Japan
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- Matsubara Hiromi
- Department of Cardiology, National Hospital Organization Okayama Medical Center, Japan
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- Yokoi Toyoharu
- Department of Diagnostic Pathology, Aichi Medical University Hospital, Japan
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- Murohara Toyoaki
- Department of Cardiology, Nagoya University Graduate School of Medicine, Japan
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Abstract
A 43-year-old man presented with dyspnea on exertion. Right heart catheterization demonstrated pulmonary arterial hypertension (PAH). He was treated with bosentan, sildenafil and intravenous epoprostenol. Despite the administration of such intensive therapy, the patient's condition deteriorated to a World Health Organization functional class (WHO-FC) of IV. He participated in a clinical trial of imatinib for PAH. After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis. At autopsy, patchy capillary proliferation was observed in the lungs. The definitive diagnosis was pulmonary capillary hemangiomatosis.<br>
Journal
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- Internal Medicine
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Internal Medicine 53 (6), 603-607, 2014
The Japanese Society of Internal Medicine