Complete response with tirabrutinib for relapsed and refractory Bing-Neel syndrome

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  • Tirabrutinibにより完全奏効を達成した再発難治性Bing-Neel症候群
  • Tirabrutinib ニ ヨリ カンゼン ソウコウ オ タッセイ シタ サイハツ ナンチセイ Bing-Neel ショウコウグン

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Description

<p>A 62-year-old female patient was diagnosed with Waldenstrom’s macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) 8 years ago, which was resolved with rituximab (R) monotherapy. Five years ago, she experienced numbness of the lower limbs, followed by diminished lower limb muscle strength and hearing disturbance. PET-CT scans showed accumulations along the peripheral nerves of the upper and lower limbs together with clonal B lymphocytes in the cerebrospinal fluid, thus a diagnosis of relapse with Bing-Neel syndrome (BNS). After a temporal remission by high-dose cytarabine or bendamustine plus R regimens as salvage treatments, WM/LPL recurred for the third time accompanied by gait disturbances due to muscle weakness and urinary retention. Thus, tirabrultinib was started as a subsequent therapy, which significantly improved the neurological condition together with abnormal findings of magnetic resonance imaging or cerebrospinal fluids. This case is valuable since few relapsed BNS was reported in the literature with successful tirabrutinib treatment.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 63 (7), 770-775, 2022

    The Japanese Society of Hematology

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