Successful treatment of Bing-Neel syndrome using combination therapy with fludarabine and rituximab

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  • NAGAHARU Keiki
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • MIYAZAMI Kana
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • IMAI Hiroshi
    Department of Pathology, Mie University Graduate School of Medicine
  • TAMURA Asako
    Department of Neurology, Mie University Graduate School of Medicine
  • UMINO Akira
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • FUJIEDA Atsushi
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • SUGIMOTO Yuka
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • YAMAGUCHI Motoko
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • MASUYA Masahiro
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • KATAYAMA Naoyuki
    Department of Hematology and Oncology, Mie University Graduate School of Medicine

Bibliographic Information

Other Title
  • フルダラビン・リツキシマブ併用療法が奏効したBing-Neel症候群
  • 症例報告 フルダラビン・リツキシマブ併用療法が奏効したBing-Neel症候群
  • ショウレイ ホウコク フルダラビン ・ リツキシマブ ヘイヨウ リョウホウ ガ ソウコウ シタ Bing-Neel ショウコウグン

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Description

Bing-Neel syndrome is known as Waldenström's macroglobulinemia with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells. A 74-year-old man was admitted because of progressive cognitive impairment. Serum immunoelectrophoresis showed a monoclonal IgM-kappa component. Bone marrow aspiration revealed 59% small lymphocytes showing plasmacytoid differentiation. Bone marrow flow cytometry disclosed a population of kappa light-chain positive lymphoid cells expressing CD19, CD20, CD38, and CD138. Magnetic resonance imaging of the brain demonstrated gadolinium-enhancement in the right temporo-parieto-occipital meninges with sulcal enhancement. Cerebrospinal fluid cytology showed a population of lymphoplasmacytoid cells, positive for CD19, CD20, CD25, and kappa light-chain. Based on these findings, Bing-Neel syndrome was diagnosed. Although combination chemotherapy consisting of intrathecal methotrexate and oral cyclophosphamide was started, his symptoms continued to worsen. Then, we initiated treatment with a regimen consisting of fludarabine/rituximab (FR). After 6 courses of this FR regimen, a complete remission was achieved. Our case suggests the FR regimen to potentially be an effective treatment option for Bing-Neel syndrome of the scattered type.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 55 (12), 2423-2428, 2014

    The Japanese Society of Hematology

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