Acute Exacerbation of Pleuroparenchymal Fibroelastosis Secondary to Allogenic Hematopoietic Stem Cell Transplantation
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- Murakami Yasushi
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan
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- Sakamoto Koji
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan
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- Okumura Yuki
- Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Japan
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- Suzuki Atsushi
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan
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- Mii Shinji
- Department of Pathology, Nagoya University Graduate School of Medicine, Japan
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- Sato Mitsuo
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan Department of Pathophysiological Laboratory Sciences, Nagoya University Graduate School of Medicine, Japan
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- Yokoi Toyoharu
- Department of Pathology, Tsushima City Hospital, Japan
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- Hashimoto Naozumi
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan
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- Hasegawa Yoshinori
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan Department of Respiratory Medicine, National Hospital Organization, Nagoya Medical Center, Japan
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Abstract
<p>In this article, we report a case with pleuroparenchymal fibroelastosis (PPFE) following hematopoietic stem cell transplantation (HSCT) that developed acute respiratory failure with new bilateral ground glass opacity, which could not be explained by either a pulmonary infection, drug toxicity or extraparenchymal causes. Although combination therapy with multiple immunosuppressants was transiently effective, the patient died from a recurrent exacerbation. Autopsied lungs demonstrated diffuse alveolar damage superimposed on PPFE. There was no evidence of any coexisting interstitial pneumonia with the usual interstitial pneumonia (UIP) pattern. Our case suggests that acute exacerbation can occur in patients with post-HSCT PPFE, even when a coexisting UIP pattern is absent. </p>
Journal
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- Internal Medicine
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Internal Medicine 59 (21), 2737-2743, 2020-11-01
The Japanese Society of Internal Medicine
