Yasuda Hajime, Tomizawa Yuji, Harada Sakiko, Sasaki Makoto, Komatsu Norio, Ando Jun, Hattori Nobutaka, Ando Miki
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
Heliyon. 2022 Oct 5;8(10):e10928. doi: 10.1016/j.heliyon.2022.e10928. eCollection 2022 Oct.
Anti-myelin-associated-glycoprotein (MAG) neuropathy is a distal, predominantly demyelinating, sensory or sensory-motor polyneuropathy most often developing in the context of an IgM-type monoclonal gammopathy due to monoclonal gammopathy of undetermined significance or lymphoplasmacytic lymphoma. Rituximab is considered standard therapy for treatment naïve patients, but optimal treatment methods for relapsed/refractory patients have not been established.
We demonstrate that tirabrutinib, a second-generation Burton kinase inhibitor, led to drastic improvements of polyneuropathy that were affirmed by nerve conduction studies in a rituximab-refractory anti-MAG neuropathy patient. Tirabrutinib continues to give excellent disease control with no apparent adverse events at 11 months since initiation, and the patient remains free of plasmapheresis sessions which were originally mandatory.
Tirabrutinib is an extremely promising treatment option for anti-MAG neuropathy.
抗髓鞘相关糖蛋白（MAG）神经病变是一种远端为主的脱髓鞘性感觉或感觉运动性多神经病，最常发生于意义未明的单克隆丙种球蛋白病或淋巴浆细胞淋巴瘤所致的IgM型单克隆丙种球蛋白病背景下。利妥昔单抗被认为是初治患者的标准治疗方法，但复发/难治性患者的最佳治疗方法尚未确立。
我们证明，第二代布鲁顿激酶抑制剂替拉鲁替尼使一名利妥昔单抗难治性抗MAG神经病变患者的多神经病得到显著改善，神经传导研究证实了这一点。自开始治疗11个月以来，替拉鲁替尼持续实现了出色的疾病控制，且无明显不良事件，该患者不再需要进行原本必需的血浆置换治疗。
替拉鲁替尼是抗MAG神经病变极具前景的治疗选择。
