Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan
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- Shimazaki Chihiro
- Department of Hematology, Japan Community Health care Organization Kyoto Kuramaguchi Medical Center, Japan
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- Hata Hiroyuki
- Department of Immunology and Hematology, Graduate School of Health Sciences, Kumamoto University, Japan
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- Iida Sinsuke
- Department of Hematology and Oncology, Nagoya City University Graduate School of Medical Sciences, Japan
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- Ueda Mitsuharu
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan
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- Katoh Nagaaki
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan
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- Sekijima Yoshiki
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan
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- Ikeda Shuichi
- Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan
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- Yazaki Masahide
- Department of Biological Sciences for Intractable Neurological Diseases, Institute for Biomedical Sciences, Shinshu University, Japan
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- Fukushima Wakaba
- Department of Public Health, Osaka City University Faculty of Medicine, Japan
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- Ando Yukio
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan
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Abstract
<p>Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. </p><p>Methods We conducted a survey of Japanese AL amyloidosis patients, who were treated between January 1, 2012, and December 31, 2014. </p><p>Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71). Diagnostic findings were most commonly detected in the GI tract (upper GI tract: 350 cases, lower GI tract: 167 cases), followed by the bone marrow and kidneys. An abdominal fat-pad biopsy was only conducted in 128 patients. Autologous stem cell transplants (ASCTs) and bortezomib were used to treat 126 and 276 patients, respectively. </p><p>Conclusion The clinical features of Japanese patients with systemic AL amyloidosis are similar to those reported previously for cases in the US and Europe. Regarding treatment, a significant number of ASCTs were performed in Japan as well as in Western countries. Surprisingly, a marked number of patients received bortezomib as a treatment for AL amyloidosis. </p>
Journal
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- Internal Medicine
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Internal Medicine 57 (2), 181-187, 2018
The Japanese Society of Internal Medicine
