正常圧水頭症で発症した軟髄膜原発B細胞性リンパ腫  [in Japanese] Primary leptomeningeal B-cell lymphoma with normal pressure hydrocephalus at diagnosis  [in Japanese]

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Author(s)

    • 石埼 卓馬 ISHIZAKI Takuma
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 野島 美久 NOJIMA Yoshihisa
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 三井 健揮 MITSUI Takeki
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 内山 由理 UCHIYAMA Yuri
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 小川 孔幸 OGAWA Yoshiyuki
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 滝沢 牧子 TAKIZAWA Makiko
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University
    • 斉藤 貴之 SAITOH Takayuki
    • 群馬大学大学院保健学研究科 生体情報検査科学 Department of Laboratory Science, Graduate School of Health Sciences, Gunma University
    • 半田 寛 HANDA Hiroshi
    • 群馬大学大学院医学系研究科 生体統御内科 Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University

Abstract

症例は80歳男性。歩行障害,物忘れで発症,頭部MRIと髄液排除試験で正常圧水頭症が疑われたが,髄液の細胞診とフローサイトメトリーの所見からB細胞性リンパ腫と診断した。頭部・脊髄MRIでは脊髄の腹側,背側表面に造影効果を認め,軟髄膜原発悪性リンパ腫(PLML)と診断。Methotrexate (MTX)髄注開始後,髄液所見,健忘症状,歩行障害は改善し,造影MRIの髄膜病変は消失した。3か月後に左顔面神経麻痺症状で再発したが,rituximab投与とMTX髄注で神経症状は消失した。その後造影MRIで脊柱管内に腫瘤性病変を認めたが,3か月ごとのMTX髄注のみで発症後4年間,神経症状なく経過している。PLMLは診断が困難で非常に稀な疾患であるが,定期的な髄注が病勢進行の抑制に有効であった。

An 80-year-old man, presenting with gait disturbance and memory loss, had findings of normal pressure hydrocephalus. Primary leptomeningeal lymphoma (PLML) was diagnosed based on cytology and flow cytometry of cerebrospinal fluid obtained by examination. Gadolinium-enhanced MRI showed enhancement of the brain and spinal cord but FDG-PET/CT revealed no lymph node swelling. With intrathecal chemotherapy, meningeal lesions disappeared and the gait disturbance and memory loss improved. However, the disease recurred three months later, manifesting as left facial nerve palsy, but the symptoms disappeared in response to intrathecal chemotherapy and systemic rituximab administration. Although a tumor lesion in the spinal canal was suggested by MRI examination, the patient has maintained a good clinical course for four years with intrathecal chemotherapy every three months. PLML is a very rare disease and its diagnosis is difficult. Repeated intrathecal chemotherapy appeared to be effective against PLML in this case.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 56(12), 2441-2446, 2015

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130005116919
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    027034258
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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