一名63岁的男子被诊断为Waldenström巨球蛋白血症(WM)。六个疗程的R-CHOP(利妥昔单抗,环磷酰胺,阿霉素,长春新碱,和泼尼松龙)导致完全缓解,但是WM在R-CHOP后三年复发。经过六个疗程的BR(苯达莫司汀,利妥昔单抗),血清IgM水平和CRP恢复正常。BR四年后,病人表现为肌肉无力,感觉障碍,和下肢肌阵挛症.T2加权磁共振成像(MRI)显示,在脑实质的右颞叶和顶叶中,信号强度高,对比度增强的区域,髓质,双侧基底神经节,枕叶白质,和胸脊髓在Th2-11水平。开放式脑活检显示脑表面和脑血管周围小淋巴细胞和浆细胞样淋巴细胞弥漫性增殖,导致Bing-Neel综合征(BNS)的诊断。两个疗程的R-MPV(利妥昔单抗,甲氨蝶呤,丙卡巴嗪,和长春新碱)导致进行性疾病,但颅脑脊髓照射30.6Gy后,神经系统症状和MRI表现有所改善。颅脑脊髓照射三年后,T2加权MRI显示BNS复发,下肢肌阵挛性进展和IgM升高。开始使用Tirabrutinib治疗第二次WM复发和BNS进展。在开始使用替拉布替尼治疗两个月后,下肢肌阵鸣消失,MRI表现好转.血清IgM水平下降,未观察到不良事件。Tirabrutinib有望作为复发性BNS的治疗选择。
A 63-year-old man was diagnosed with Waldenström\'s macroglobulinemia (WM). Six courses of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) resulted in complete remission, but WM relapsed three years after R-CHOP. After six courses of BR (bendamustine, rituximab), the serum IgM level and CRP normalized. Four years after BR, the patient presented with muscle weakness, sensory disturbance, and myoclonus of lower limbs. T2-weighted magnetic resonance imaging (MRI) showed areas of signal hyperintensity with contrast enhancement in the right temporal and parietal lobes in brain parenchyma, medulla, bilateral basal ganglia, white matter of occipital lobe, and thoracic spinal cord at the Th2-11 levels. Open brain biopsy revealed diffuse proliferation of small lymphocytes and plasmacytoid lymphocytes on the brain surface and around cerebral blood vessels, resulting in a diagnosis of Bing-Neel syndrome (BNS). Two courses of R-MPV (rituximab, methotrexate, procarbazine, and vincristine) resulted in progressive disease, but the neurological symptoms and MRI findings improved following craniospinal irradiation of 30.6 Gy. Three years after craniospinal irradiation, T2-weighted MRI showed recurrence of BNS with progression of myoclonus of lower limbs and IgM elevation. Tirabrutinib was started for the second recurrence of WM and progression of BNS. Two months after the initiation of treatment with tirabrutinib, the myoclonus of lower limbs disappeared and the MRI findings showed improvement. Serum IgM levels decreased and no adverse events were observed. Tirabrutinib shows promise as a therapeutic option for relapsed BNS.