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- W2995070137 abstract "Objective: To improve the understanding of rare anti-myelin-associated glycoprotein (MAG) positive IgM monoclonal gammopathy related peripheral neuropathy (IgM-PN) . Methods: Eleven cases of IgM paraproteinemia and anti-MAG antibody positive neuropathy diagnosed since 2014 in Peking Medical Union College Hospital were summarized. The medical records including clinical manifestation, lab results, treatment and prognosis were analyzed. Results: Among the 11 patients (8 male and 3 female) , the median onset age is 63 years old (range from 52 to 77 years old) . The peripheral neuropathy of 9 patients were characterized by distal onset of numbness, 6 patients suffered from muscle weakness. The nerve conduction velocity study indicated that all 11 patients had demyelinating peripheral nerve damage, which was sensory predominant and more severe in lower limbs, 6 of them had secondary axonal damage. Monoclonal IgM gammopathy was identified in all 11 patients, among which 6 were IgM κ, 2 IgG κ and IgM κ bi-clonal, 3 IgM λ. Three patients were diagnosed with Waldenström's macroglobulinaemia. The anti-MAG-IgM antibody was positive in all 11 cases. After diagnosis, 9 patients received combination chemotherapy including rituximab or rituximab treatment alone. The monoclonal IgM level declined significantly in 7 patients. The neuropathy was stable or improved. Conclusions: Anti-MAG antibody positive IgM-PN is a rare M protein related disease. In peripheral neuropathy with undetermined etiology, we suggest to screen M protein and anti-MAG antibody. Chemotherapy including rituximab or rituximab alone is recommended as first-line therapy.目的： 提高对罕见的抗髓鞘相关糖蛋白（MAG）抗体阳性的IgM相关性周围神经病（IgM-PN）的认识。 方法： 总结2014年1月至2019年4月北京协和医院诊断的11例抗MAG抗体阳性的IgM-PN患者的临床特点、实验室检查、治疗方案和预后。 结果： 11例患者中，男8例，女3例，中位发病年龄63（52~77）岁。其中9例患者以远端肢体麻木起病，6例伴肌力减退。神经传导速度检查示，均为周围神经脱髓鞘损害，以下肢感觉神经损害为主，6例伴慢性轴索损害。11例患者均存在血清IgM型单克隆免疫球蛋白，6例为IgM κ型，3例为IgM λ型，2例为IgM κ/IgG κ双克隆型。3例患者继发于巨球蛋白血症。11例患者的血清抗MAG抗体均为阳性。9例患者接受利妥昔单抗单药或联合化疗，治疗后7例患者的神经症状稳定或改善。 结论： 抗MAG抗体阳性的IgM-PN是一种罕见的M蛋白相关性疾病。对于伴IgM型M蛋白的周围神经病患者，应常规筛查抗MAG抗体。基于利妥昔单抗的治疗可作为其一线治疗方案。." @default.
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- W2995070137 date "2019-11-14" @default.
- W2995070137 modified "2023-09-25" @default.
- W2995070137 title "[Anti-myelin-associated glycoprotein antibody positive IgM monoclonal gammopathy related peripheral neuropathy: 11 cases and literature review]." @default.
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- W2995070137 doi "https://doi.org/10.3760/cma.j.issn.0253-2727.2019.11.003" @default.
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