Department of Neurology, Wayne State University-Detroit Medical Center, 4201 St. Antoine UHC 8C.28, Detroit, MI 48201, USA.
Curr Neurol Neurosci Rep. 2012 Feb;12(1):102-10. doi: 10.1007/s11910-011-0237-4.
Peripheral neuropathy associated with monoclonal gammopathy is a rare but important cause of neuropathy that can herald serious underlying disease. IgM monoclonal gammopathy of undetermined significance (MGUS) is the most commonly found monoclonal gammopathy associated with neuropathy, with characteristic clinical, electrophysiologic, and pathologic features. The IgG and IgA monoclonal gammopathies are rarely associated with specific neuropathies. Standard immunomodulatory agents including steroids, intravenous immunoglobulin, and plasmapheresis have shown limited efficacy in IgM MGUS. Neuropathies associated with specific lymphoproliferative disorders may not respond to treatments aimed at that disorder. Case series had shown promising results with rituximab, a monoclonal antibody that targets the B cell surface antigen CD20 and results in a rapid and sustained depletion of B cells; however, two recent randomized controlled trials with rituximab failed to provide evidence of efficacy in primary outcome measures, despite reduction in antibody levels. Long-term studies looking at the association between specific immunologic markers and disease recurrence are needed to ultimately develop targeted therapies.
与单克隆丙种球蛋白血症相关的周围神经病是一种罕见但重要的神经病病因，可能预示着潜在的严重疾病。意义未明的单克隆丙种球蛋白血症（MGUS）是最常见的与神经病相关的单克隆丙种球蛋白血症，具有特征性的临床、电生理和病理特征。IgG 和 IgA 单克隆丙种球蛋白病很少与特定的神经病相关。包括类固醇、静脉注射免疫球蛋白和血浆置换在内的标准免疫调节药物在 IgM MGUS 中的疗效有限。与特定淋巴增生性疾病相关的神经病可能对针对该疾病的治疗无反应。病例系列研究显示，利妥昔单抗（一种针对 B 细胞表面抗原 CD20 的单克隆抗体）具有有前途的结果，可导致 B 细胞快速和持续耗竭；然而，两项最近的利妥昔单抗随机对照试验未能提供疗效的证据，尽管抗体水平降低。需要进行长期研究，以确定特定免疫标志物与疾病复发之间的关联，最终开发靶向治疗。
