Hagihara Masao, Ide Shiro, Ohara Shin, Imai Yui, Uchida Tomoyuki, Inoue Morihiro
Department of Hematology, Eiju General Hospital.
Rinsho Ketsueki. 2022;63(7):770-775. doi: 10.11406/rinketsu.63.770.
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.
一名62岁女性患者8年前被诊断为华氏巨球蛋白血症/淋巴浆细胞淋巴瘤（WM/LPL），通过利妥昔单抗（R）单药治疗得到缓解。5年前，她出现下肢麻木，随后下肢肌肉力量减弱和听力障碍。PET-CT扫描显示上肢和下肢周围神经有积聚，同时脑脊液中有克隆性B淋巴细胞，因此诊断为伴宾-尼尔综合征（BNS）复发。在通过大剂量阿糖胞苷或苯达莫司汀加R方案作为挽救治疗获得暂时缓解后，WM/LPL第三次复发，伴有因肌肉无力和尿潴留导致的步态障碍。因此，开始使用替拉布替尼作为后续治疗，该治疗显著改善了神经状况以及磁共振成像或脑脊液的异常表现。该病例很有价值，因为文献中很少报道复发的BNS经替拉布替尼治疗成功的案例。
