Traub Rebecca, Rubinstein Samuel M, Dittus Christopher
University of North Carolina, Chapel Hill, 170 Manning Drive, Chapel Hill, NC, 27599-7025, USA.
Curr Neurol Neurosci Rep. 2025 Jun 23;25(1):43. doi: 10.1007/s11910-025-01430-w.
This article reviews recent clinical updates and research on the evaluation and management of patients with peripheral neuropathy in association with monoclonal gammopathies.
Recent studies have elucidated pathogenic mechanisms of IgM paraprotein associated neuropathies, including nodal and paranodal targets and complement mediated processes, suggesting novel therapeutic targets. New chemotherapeutic regimens have improved outcomes and neurotoxic side effect profiles in the treatment of patients with light chain (AL) amyloidosis and POEMS syndrome. Establishing when a monoclonal gammopathy is causative of a peripheral neuropathy remains a clinical challenge. New therapeutic drugs for treatment of IgM paraprotein associated neuropathies show promise. Identifying AL amyloidosis and POEMS early in patients presenting with neuropathy are important. Clinical phenotyping and antibody testing are critical to evaluating patients with paraproteins and peripheral neuropathy.
本文综述了近期关于单克隆丙种球蛋白病相关周围神经病患者评估与管理的临床进展及研究。
近期研究阐明了IgM副蛋白相关神经病的致病机制，包括结区和旁结区靶点以及补体介导的过程，提示了新的治疗靶点。新的化疗方案改善了轻链（AL）淀粉样变性和POEMS综合征患者的治疗效果及神经毒性副作用情况。确定单克隆丙种球蛋白病何时导致周围神经病仍是一项临床挑战。治疗IgM副蛋白相关神经病的新治疗药物显示出前景。在出现神经病的患者中早期识别AL淀粉样变性和POEMS很重要。临床表型分析和抗体检测对于评估副蛋白血症和周围神经病患者至关重要。
