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

  • Guo Yong-mei
    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
  • 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

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 58 (20), 3039-3043, 2019-10-15

    The Japanese Society of Internal Medicine

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