Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency

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Author(s)

    • Guo Yong-mei
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Goto Akiteru
    • Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
    • Takahashi Naoto
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Takahashi Nagi
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Miyabe Ken
    • Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
    • Yoshida Makoto
    • Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan
    • Abe Fumito
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Yamashita Takaya
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Nara Miho
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Yoshioka Tomoko
    • Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
    • Ohashi Kenichi
    • Department of Pathology, Yokohama City 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

  • Internal Medicine

    Internal Medicine, 2020

    The Japanese Society of Internal Medicine

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