Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency
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- Guo Yong-mei
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Takahashi Nagi
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Miyabe Ken
- Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
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- Yoshida Makoto
- Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
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- Abe Fumito
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Yamashita Takaya
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Nara Miho
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Yoshioka Tomoko
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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- Ohashi Kenichi
- Department of Pathology, Yokohama City University Graduate School of Medicine, Japan
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- Goto Akiteru
- Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
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- Takahashi Naoto
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Abstract
<p>Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfunction two weeks after admission. The diagnosis was confirmed histologically by AL-λ amyloidosis, with the liver and spleen as the main lesions, on an autopsy. As treatment-related toxicity is strong in advanced cases, appropriate treatments are required to improve the prognosis of AL amyloidosis with severe liver dysfunction. </p>
Journal
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- Internal Medicine
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Internal Medicine 58 (20), 3039-3043, 2019-10-15
The Japanese Society of Internal Medicine