VA Pittsburgh Healthcare System, MSSL-Neurology, Pittsburgh, PA, USA.
Leuk Lymphoma. 2009 Sep;50(9):1422-33. doi: 10.1080/10428190903111922.
Monoclonal proteins (paraproteins) may be detected in the sera in approximately 1% of the general population and are frequently associated with peripheral neuropathy. Paraproteinemic neuropathy (PPN) is most commonly associated with monoclonal gammopathy of undetermined significance, and may also occur in the context of multiple myeloma, amyloidosis, cryoglobulinemia, and other hematologic malignant and nonmalignant conditions. Possible malignant conversion of underlying benign monoclonal gammopathy should be closely monitored, and risk factors include progression of neuropathy and rising titers of monoclonal protein. PPN is frequently associated with autoantibodies targeting peripheral nerve antigens, including anti-MAG and anti-GM1 antibodies. Therapy is mostly based on the treatment of the underlying hematologic disorder. Risks and benefits should be carefully evaluated as treatment may be associated with considerable morbidity. Rituximab may be helpful in treatment of IgM-PPN, and paraproteinemic multifocal motor neuropathy usually responds to IVIG. Plasmapheresis is more effective with IgG/IgA paraproteinemic neuropathies. At this time it is not clear whether mere presence of neuropathy warrants more aggressive treatment of otherwise quiescent hematologic malignances (e.g. smoldering myeloma).
单克隆蛋白（副蛋白）在约 1%的普通人群的血清中可被检测到，且常与周围神经病相关。副蛋白血症性多发性神经病（PPN）最常与意义未明的单克隆丙种球蛋白血症相关，也可发生于多发性骨髓瘤、淀粉样变性、冷球蛋白血症及其他血液恶性和非恶性疾病中。应密切监测潜在良性单克隆丙种球蛋白的恶性转化可能，且危险因素包括神经病进展和单克隆蛋白滴度升高。PPN 常与针对周围神经抗原的自身抗体相关，包括抗髓鞘相关糖蛋白（MAG）抗体和抗神经节苷脂 GM1 抗体。治疗主要基于基础血液疾病的治疗。应仔细评估风险和获益，因为治疗可能与相当大的发病率相关。利妥昔单抗可能对 IgM-PPN 有帮助，而副蛋白血症性多灶性运动神经病通常对 IVIG 有反应。对于 IgG/IgA 副蛋白血症性神经病，血浆置换更有效。目前尚不清楚是否存在神经病变就需要对原本静止的血液恶性肿瘤（如冒烟型骨髓瘤）进行更积极的治疗。
