Recurrence of Waldenström macroglobulinemia accompanied by factor X deficiency

  • OHARA Shin
    Department of Hematology, Eiju General Hospital
  • HAGIHARA Masao
    Department of Hematology, Eiju General Hospital
  • HUA Jian
    Department of Hematology, Eiju General Hospital
  • INOUE Morihiro
    Department of Hematology, Eiju General Hospital
  • UCHIDA Tomoyuki
    Department of Hematology, Eiju General Hospital
  • YOSHINAGA Tsuneaki
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • YAZAKI Masahide
    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
  • SEKIJIMA Yoshiki
    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
  • KAMETANI Fuyuki
    Tokyo Metropolitan Institute of Medical Science

Bibliographic Information

Other Title
  • 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
  • 症例報告 第2回日本血液学会関東甲信越地方会 優秀演題 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
  • ショウレイ ホウコク ダイ2カイ ニホン ケツエキ ガッカイ カントウ コウシンエツチホウカイ ユウシュウ エンダイ ダイX インシ ケツボウショウ オ トモナッテ サイネン シタ ゲンパツセイ マクログロブリン ケツショウ

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Abstract

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

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