第X因子欠乏症を伴って再燃した原発性マクログロブリン血症  [in Japanese] Recurrence of Waldenström macroglobulinemia accompanied by factor X deficiency  [in Japanese]

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Author(s)

    • 華 見 HUA Jian
    • 永寿総合病院 血液内科 Department of Hematology, Eiju General Hospital
    • 吉長 恒明 YOSHINAGA Tsuneaki
    • 信州大学医学部 脳神経内科,リウマチ・膠原病内科 Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
    • 矢崎 正英 YAZAKI Masahide
    • 信州大学医学部 脳神経内科,リウマチ・膠原病内科|信州大学 バイオメディカル研究所 Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine|Department of Biological Sciences for Intractable Neurological Disorders, Institute for Biomedical Sciences, Shinshu University
    • 関島 良樹 SEKIJIMA Yoshiki
    • 信州大学医学部 脳神経内科,リウマチ・膠原病内科|信州大学 バイオメディカル研究所 Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine|Department of Biological Sciences for Intractable Neurological Disorders, Institute for Biomedical Sciences, Shinshu University

Abstract

症例は85歳,男性。11年前に原発性マクログロブリン血症と診断され,cyclophosphamide内服での加療歴があった。今回健診で貧血を指摘され,原病の増悪と判断され入院,RCD (rituximab, cyclophosphamide, dexamethasone)療法が開始された。一方,入院時の採血でPT, APTT値延長を認め,精査の結果第X因子欠乏合併と診断された。治療効果が得られないまま,第154病日に自宅で急変し永眠された。病理組織所見やアミロイド蛋白のシークエンス解析により,ALアミロイドーシス合併が判明した。原発性マクログロブリン血症にALアミロイドーシスを合併し,さらに第X因子欠乏を伴って再燃した例は稀であり報告する。

A medical check-up revealed severe anemia in an 85-year-old man who had been diagnosed with Waldenström macroglobulinemia 11 years previously. On the other hand, prolonged PT and aPTT were demonstrated on admission, and were attributed to a significant decrease in factor X activity. These abnormalities were all considered to be have been caused by an exacerbation of the underlying disease and, thus, chemotherapy with the RCD regimen (rituximab, cyclophosphamide, dexamethasone) was started. No significant improvement was obtained and the patient died suddenly on day 154. AL amyloidosis was diagnosed by histopathological examinations and also confirmed by a sequence analysis of amyloid protein. This case with Waldenström macroglobulinemia complicated by AL amyloidosis and recurrent factor X deficiency is quite rare.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 57(3), 359-363, 2016

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130005145489
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    027245775
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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