A CASE OF PRIMARY AMYLOIDOSIS WITH RAPIDLY PROGRESSIVE MULTIPLE ORGAN FAILURE

  • HONDA DAISUKE
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • IZUMI YUKO
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • GOHDA TOMOHITO
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • NAGAMACHI SEIJI
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • OHSAWA ISAO
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • HORIKOSHI SATOSHI
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • SEKINE RYO
    DIVISION OF GASTROENTEROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • KURISAKI AIKO
    DEPARTMENT OF HUMAN PATHOLOGY, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • WATANABE SUMIO
    DIVISION OF GASTROENTEROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • YAO TAKASHI
    DEPARTMENT OF HUMAN PATHOLOGY, JUNTENDO UNIVERSITY FACULTY OF MEDICINE
  • TOMINO YASUHIKO
    DIVISION OF NEPHROLOGY, DEPARTMENT OF INTERNAL MEDICINE, JUNTENDO UNIVERSITY FACULTY OF MEDICINE

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Other Title
  • 急速に進行する多臓器不全をきたした原発性アミロイドーシスの1剖検例
  • 症例報告検討会 急速に進行する多臓器不全をきたした原発性アミロイドーシスの1剖検例
  • ショウレイ ホウコク ケントウカイ キュウソク ニ シンコウ スル タゾウキ フゼン オ キタシタ ゲンパツセイ アミロイドーシス ノ 1 ボウケンレイ

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We report a case of a 71-year-old man with various symptoms, including appetite loss, sensory disorder of taste, diarrhea and a decrease of body weight. He also presented hepatosplenomegaly, anemia, renal dysfunction and hypoalbuminemia. Although he was diagnosed with systemic amyloidosis through rectal and abdominal fat biopsies, he showed rapidly progressive multiple organ failure, which resulted in death. His autopsy findings contributed to a final diagnosis of primary amyloidosis, caused by monoclonal gammopathy of undetermined significance (MGUS). 1-1.5% of MGUS patients progress to multiple myeloma, amyloidosis, or some other more acute disease within a year. It is important to make a differential diagnosis, including primary amyloidosis, for patients with various symptoms. Even if the patient has a normal level of serum IgG, we need to pay attention to MGUS, as it can progress to multiple organ failure.

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