IgG-variant Bing-Neel syndrome diagnosed by detecting <i>MYD88</i> L265P mutation in the cerebrospinal fluid cells

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  • MARUYAMA Yumiko
    Department of Hematology, University of Tsukuba Hospital
  • NISHIKII Hidekazu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • MATSUOKA Ryota
    Department of Pathology, Faculty of Medicine, University of Tsukuba
  • MAKISHIMA Kenichi
    Department of Hematology, University of Tsukuba Hospital
  • KURITA Naoki
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • KUSAKABE Manabu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • YOKOYAMA Yasuhisa
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • KATO Takayasu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • SAKATA-YANAGIMOTO Mamiko
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • OBARA Naoshi
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • NAKAMURA Naoya
    Department of Pathology, Tokai University School of Medicine
  • CHIBA Shigeru
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba

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Other Title
  • 髄液細胞の<i>MYD88</i> L265P変異により診断に至ったIgG型Bing-Neel症候群
  • 髄液細胞のMYD88 L265P変異により診断に至ったIgG型Bing-Neel症候群
  • ズイエキ サイボウ ノ MYD88 L265P ヘンイ ニ ヨリ シンダン ニ イタッタ IgGガタ Bing-Neel ショウコウグン

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Abstract

<p>Bing-Neel syndrome (BNS), which presents with a variety of neurological complications, is a rare manifestation of the lymphoplasmacytic lymphoma (LPL) and is characterized by the infiltration of LPL cells into the central nervous system. In this study, we report the case of a patient with BNS, which was confirmed by detecting MYD88 L265P mutation in the cerebrospinal fluid (CSF) cells. A 74-year-old patient was diagnosed with IgG-variant LPL. He achieved a very good partial response to the treatment with rituximab and bendamustine (RB) and was stable for over 5 years, when presenting a slowly progressive motor deficit in the lower limbs. It was difficult to confirm BNS from morphological analysis of the CSF cells. After detecting MYD88 L265P mutation in the CSF cells, he was subsequently diagnosed with BNS and treated with RB and intrathecal chemotherapy, resulting in rapid clinical improvement. With the onset of neurological manifestation during the clinical course of LPL, the detection of MYD88 L265P mutation in the CSF cells could be helpful for the diagnosis and management of BNS.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 62 (10), 1493-1498, 2021

    The Japanese Society of Hematology

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