Gao X M, Jia M N, Qian M, Ren H T, Zhang L, Shen K N, Cao X X, Li J
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China.
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Nov 14;40(11):901-905. doi: 10.3760/cma.j.issn.0253-2727.2019.11.003.
To improve the understanding of rare anti-myelin-associated glycoprotein (MAG) positive IgM monoclonal gammopathy related peripheral neuropathy (IgM-PN) . 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. 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. 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.
为提高对罕见的抗髓鞘相关糖蛋白（MAG）阳性IgM单克隆丙种球蛋白病相关周围神经病（IgM-PN）的认识。总结了自2014年以来在北京协和医院诊断的11例IgM副蛋白血症和抗MAG抗体阳性神经病患者。分析了包括临床表现、实验室检查结果、治疗及预后等方面的病历资料。11例患者（8例男性，3例女性）中，发病年龄中位数为63岁（范围52至77岁）。9例患者的周围神经病以远端麻木起病为特征，6例患者有肌无力。神经传导速度检查显示，11例患者均有脱髓鞘性周围神经损害，以感觉为主，下肢更严重，其中6例有继发性轴索损害。11例患者均检测到单克隆IgM丙种球蛋白病，其中6例为IgM κ型，2例为IgG κ型及IgM κ双克隆型，3例为IgM λ型。3例患者诊断为华氏巨球蛋白血症。11例患者抗MAG-IgM抗体均为阳性。诊断后，9例患者接受了包括利妥昔单抗的联合化疗或单独使用利妥昔单抗治疗。7例患者单克隆IgM水平显著下降。周围神经病病情稳定或改善。抗MAG抗体阳性IgM-PN是一种罕见的M蛋白相关疾病。对于病因不明性周围神经病，建议筛查M蛋白和抗MAG抗体。推荐以利妥昔单抗或单独使用利妥昔单抗的化疗作为一线治疗方案。
