口腔内血腫を初発症状とした原発性アミロイドーシスの1例

  • 新美 直哉
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学
  • 重冨 俊雄
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学
  • 大野 雄弘
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学
  • 小田 有紀子
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学
  • 水谷 英樹
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学
  • 上田 実
    名古屋大学大学院医学研究科頭頸部・感覚器外科学講座顎顔面外科学

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  • A case of primary amyloidosis with incipient symptoms of hematoma in the oral cavity.

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Amyloid is defined as a pathologic fibrous proteinaceous substance which, when deposited between the cells of tissues and organs, leads to various clinical conditions. Immunohistochemistry has allowed for better classification and understanding of the pathophysiology of amyloidosis.<BR>A 70-year-old man was referred to our department because of spontaneous purpura of the skin and hematoma of the oral mucosa. There was no chemical or hematologic abnormality on routine examination. Bence Jones protein was positive in the urine. Bone marrow examination, however, revealed an slightly increased proportion of plasma cells. It was unlikely that the Bence Jones protein was associated with multiple myeloma. Skin biopsy revealed depositions of amyloid, and Congo red stain for amyloid was positive. In addition, immunofluoroecent staining resulted in a diagnosis of AA amyloidosis.<BR>The patient was treated with a combination of colchicine, melphalan, and prednisolone; however, 7 months after initial presentation he died of cardiac failure. Autopsy revealed multiple-organ vascular amyloidosis, and a diagnosis of bullous amyloidosis was made.

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